TEXAS OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE CWD TSE PRION April 2019 a Review
February 17, 2023 TEXAS CWD COUNT AT 449 CASES TO DATE
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
The task of managing disease in cervids (Cervidae, members of the deer family) is shared by the Texas Animal Health Commission and the Texas Parks and Wildlife Department. The agencies’ performance of this task has been scrutinized following the discovery of chronic wasting disease, a neurological disease affecting cervids, in a Texas deer- breeding facility in June 2015. The discovery prompted new regulation, which representatives of the $349.4 million deer- breeding industry say has been more harmful to the industry than the disease has been. The state agencies maintain that the rules are necessary to decrease the probability of chronic wasting disease being spread from facilities where it might exist and to increase the probability of detecting and containing chronic wasting disease in facilities where it does exist. This overview, which was prepared at the request of members of the Legislature, shows the state’s response to chronic wasting disease, including agency authority, activities, and expenditures.
FACTS AND FINDINGS
The Parks and Wildlife Department has primary responsibility to protect the state’s fish and wildlife resources, which includes disease management efforts for the state’s native cervid species, white-tailed and mule deer.
The Animal Health Commission has primary responsibility for managing and responding to diseases and pests of consequence that affect nonnative cervid species, which includes elk, moose, and others. The Animal Health Commission also coordinates disease-control efforts for native cervids and works collaboratively with the Parks and Wildlife Department in that area.
Although both state agencies monitor and respond to a number of diseases affecting cervids, chronic wasting disease has been a significant focal point in recent years for the agencies and for stakeholders. Chronic wasting disease is unique relative to other diseases affecting cervids because it invariably is fatal, has a long incubation period, and virtually is impossible to eradicate.
The current chronic wasting disease regulatory structure for white-tailed and mule deer was initiated in June 2016. This structure mandates certain testing requirements and restrictions on the artificial movement of deer. It was devised using a facilitated negotiation process with stakeholders, including representatives of the deer-breeding industry.
The artificial movement of cervids increases risks for disease management, but it is a key component of the deer breeding industry and overall deer management.
From fiscal years 2011 to 2017, the Parks and Wildlife Department reports expending approximately 3.1 percent ($4.5 million) of its appropriations under Strategy A.1.1, Wildlife Conservation, for purposes related to chronic wasting disease. From fiscal years 2005 to 2017, the Animal Health Commission reports expending approximately 1.2 percent ($2.1 million) of its agencywide appropriations for purposes related to chronic wasting disease.
Legislative Budget Board staff found no indications that the collaboration between the Animal Health Commission and the Parks and Wildlife Department results in duplication of effort, nor that either agency exceeds its scope of authority or fails to engage stakeholders adequately in response to the disease.
DISCUSSION
According to the Texas Parks and Wildlife Department (TPWD), Texas is home to from 3.5 million to 4.5 million white-tailed and mule deer, which are the only cervids native to the state. Approximately 100,000 to 110,000 deer also are held in captivity as part of the state’s deer-breeding industry.
Populations of other free-ranging and captive cervids in the state, such as elk, red deer, and sika, are not native to Texas and are much smaller in number compared to white-tailed and mule deer. State law deems cervids and all other wild animals inside the borders of the state the property of the people of the state, regardless of whether the movement of those animals is restricted by the existence of a fence constructed or maintained by a landowner. The law grants TPWD primary responsibility for protecting the state’s wildlife resources and requires the Texas Animal Health
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OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
Commission (TAHC) to protect all livestock and exotic livestock from diseases recognized as communicable by the veterinary profession.
The deer-breeding industry is a prominent part of the community regulated by TPWD and TAHC. Deer breeding had a direct economic impact of $349.4 million in 2015, as estimated by the Agricultural and Food Policy Center at Texas A&M University. That estimate increases to $1.6 billion when considering indirect impacts, such as purchases of feed and veterinary supplies, and the economic impact of deer hunting stemming from breeding operations. The production side of the deer-breeding industry typically consists of operations involved in breeding and raising deer; the consumption side typically consists of other industry breeders and hunting operations such as game ranches. Hunting is the primary end market that the industry services, and many industry producers selectively breed deer to attain genetic characteristics desirable to hunters, namely, trophy antler racks. Deer-breeding operations vary, but often involve a certain amount of fenced acreage, with a subset dedicated to breeding pens, where deer are bred, nursed, provided supplemental feed and veterinary care, tagged for identification, and ultimately sold. Sales involve the transfers of deer, which are conducted with TPWD permits.
CHRONIC WASTING DISEASE
Cervids are susceptible to multiple diseases, including chronic wasting disease (CWD), anthrax, tuberculosis, epizootic hemorrhagic disease, pneumonia, and bluetongue. TPWD and TAHC monitor these diseases in the state’s cervid population—the former in its capacity as protector of the state’s fish and wildlife resources and the latter in its capacity as protector of all livestock and exotic livestock from communicable disease.
TPWD and TAHC report being concerned about CWD before it was discovered in Texas. The disease first was identified in captive mule deer in Colorado in 1967 and later was classified as a transmissible spongiform encephalopathy, or prion disease, which is a family of rare progressive neurodegenerative disorders that can affect, separately, humans and animals. Other animal prion diseases include bovine spongiform encephalopathy, better known as mad cow disease, and scrapie, which affects sheep and goats. The term prions refers to abnormal, pathogenic agents that are transmissible and able to induce abnormal folding of specific normal cellular proteins that are found most abundantly in the brain. The abnormal folding leads to brain damage and the characteristic signs and symptoms of the disease. Those symptoms include drastic weight loss (or wasting), stumbling, and listlessness, which can render CWD-positive animals vulnerable to other mortality factors separate from the disease, such as predation and vehicle collisions.
CWD has spread steadily and has been reported in 25 states in the continental U.S. and two Canadian provinces, as shown in Figure 1. Although the overall occurrence of CWD in free-ranging deer and elk is relatively low nationwide, infection rates greater than one in 10 have been found in locations where the disease is established. Infection rates in some captive herds can be much higher, with a rate of 79.0 percent reported within one captive herd. In that case, CWD was diagnosed in a white-tailed deer from a captive farm in Wisconsin, after which the farm was quarantined and then depopulated more than four years later. Sixty of the 76 animals at the time of depopulation were found to be positive for the CWD-associated prion.
CWD presents stakeholders with challenges unlike other diseases affecting cervids. It is fatal and has no treatments or vaccines. CWD has a long incubation period of a reported minimum of approximately 17 months, with an unknown maximum. It is not known when during the course of infection an animal may be infectious. It is believed that CWD prions likely spread among animals through bodily fluids, either through direct contact or indirectly through environmental contamination of soil, food, or water. No known management strategies are available to mitigate the risk of indirect transmission of CWD when an environment has been contaminated, which makes eradication of the disease difficult, if not impossible, in areas where CWD has been long established before detection.
Given these characteristics, the risk of inadvertently spreading CWD is highest during the artificial movement of deer by human transport. In such a scenario an infected or exposed animal, whether it is a breeder deer or a trapped free-ranging deer, could be transported across the state in a trailer and disperse the disease into additional captive or free-ranging populations that otherwise would have been impossible given the deer’s natural movement patterns.
To date, no cases of CWD infection have been reported in people. However, animal studies have suggested that CWD poses a risk to some types of nonhuman primates that eat meat from CWD-infected animals or come in contact with brain or bodily fluids from infected deer or elk. The Centers for Disease Control and Prevention advises against handling
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FIGURE 1
CHRONIC WASTING DISEASE DETECTED IN NORTH AMERICA AUGUST 2018
States and provinces where Chronic Wasting Disease has been detected
Sources: Legislative Budget Board; Centers for Disease Control and Prevention.
or eating meat from deer and elk that look sick or are acting strangely or that are found dead.
AGENCY SCOPES OF AUTHORITY
The Texas Parks and Wildlife Code provides TPWD varied authority regarding the management of cervids, including the authority to take or manage native cervids for disease diagnosis or prevention; the authority to regulate the means, methods, and places in which it is lawful to hunt, take, or possess game animals; and the authority to regulate the conditions within which a person may possess a live native cervid with a TPWD-issued permit. Figure 2 shows a comparison of TPWD’s deer-related permits.
The Texas Agriculture Code, Section 161.041, requires TAHC to protect livestock from communicable disease and authorizes the agency to “act to eradicate or control any disease or agent of transmission for any disease that affects livestock, exotic livestock, domestic fowl, or exotic fowl, regardless of whether the disease is communicable, even if the agent of transmission is an animal species that is not subject to the jurisdiction of TAHC.” This latter condition includes the native cervid species of white-tailed and mule deer, which are within TPWD’s jurisdiction, because the statutory definition of exotic livestock includes only nonnative animals from the deer family. Statute addresses the overlap, however, by prohibiting TAHC from infringing on or superseding the authority of any other state agency, including TPWD’s authority relating to wildlife. Statute also requires TAHC to assume responsibility for disease control efforts if a conflict of authority exists, but to work collaboratively with the other state agency—TPWD, in this case—to enable each agency to carry out its responsibilities effectively.
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OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
FIGURE 2
TEXAS PARKS AND WILDLIFE DEPARTMENT DEER-RELATED PERMITS FISCAL YEAR 2018
PERMIT
Deer breeder permit
Deer Management Permit (DMP)
Trap, Transplant, and Transport (TTT) permit
Trap, Transplant, and Process (TTP) permit
Scientific research permit
Zoological research permit
PURPOSE
Authorizes individuals to hold white-tailed and mule deer in captivity for the purpose of propagation
Authorizes owners of high-fenced properties to detain white-tailed deer temporarily in breeding pens located on the property for the purpose of natural breeding
Authorizes municipalities, political subdivisions, and certain qualified individuals to trap white-tailed and mule deer on properties with excess population numbers and to relocate the deer to properties with sufficient habitat to support the additional animals
Authorizes cities, towns, villages, counties, special districts, property owners associations, and certain qualified individuals to capture surplus deer, process their carcasses, and donate the resulting venison to penal facilities or charitable organizations for human consumption
Authorizes employees or representatives of certain entities to collect, salvage, band, or hold native Texas wildlife for scientific purposes
Authorizes agents of certain facilities to hold native wildlife to further scientific understanding of protected wildlife, encourage management and conservation of protected wildlife, or further awareness and understanding of the biology of protected wildlife
FEE
$200 $1,000 $750 per release site $0 $53 (unless exempt) $158
Source: Texas Parks and Wildlife Department.
AGENCY ACTIVITIES
TAHC and TPWD collaborate on cervid disease management in many ways, with most examples related to the management of CWD. The agencies co-chair the CWD Task Force, which was established in 2006 to work with public and private stakeholders in developing rules and monitoring and managing CWD-related issues. TPWD provides biological information and statistics for native and nonnative species, and TAHC provides epidemiological expertise. Both agencies approve herd plans, which are requirements for disease testing and management established for deer-breeding facilities that have CWD-positive animals. Both agencies also coordinate to develop containment and surveillance zones in and around areas that have CWD and to train agency staff and others to collect samples for disease testing. Agency definitions of containment and surveillance zones terms vary slightly, but containment zones typically are geographic areas within which CWD has been detected or detection is probable, and surveillance zones are geographic areas within which the presence of CWD could reasonably be expected. The artificial movement of deer is restricted in both types of zones, and hunters who harvest CWD-susceptible species in either are required to bring their animals to a TPWD check station within 48 hours for testing.
Independently of TAHC, TPWD monitors disease in cervid and other wildlife populations by investigating reports of sick animals and mortalities. TPWD also tests roadkill, deer exhibiting clinical symptoms of disease, and hunter-harvested deer throughout the state. Independently of TPWD, TAHC’s role varies based on the disease, but typically includes the following actions:
• surveillance, which consists of varying levels of disease testing to detect presence of a disease, assess its spatial distribution and prevalence, and monitor changes in prevalence and direction of spread or contraction;
• reporting;
• setting testing and record-keeping requirements;
• epidemiological investigations to determine the disease source and exposure;
• issuing movement restrictions such as hold orders and quarantines;
• developing herd plans;
• conducting records and premises inspections;
• assisting the U.S. Department of Agriculture (USDA) to gather data for potential federal indemnification of affected herds or animals;
• proposing and establishing Texas entry requirements and disease risk zones; and
• enforcing all TAHC cervid regulations.
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FIGURE 3
TEXAS PARKS AND WILDLIFE DEPARTMENT CHRONIC WASTING DISEASE-RELATED EXPENDITURES FISCAL YEARS 2011 TO 2017
Source: Texas Parks and Wildlife Department.
TAHC also sets entry requirements for nonnative cervids. The agency also administers Texas’ voluntary native and nonnative herd certification programs for CWD, tuberculosis, and brucellosis and the Certified CWD Postmortem Sample Collector Authorized Personnel Program, which trains nonveterinarians to collect and submit samples for official post-mortem CWD testing in Texas.
ACTIVITIES IN OTHER STATES
Texas is joined by the other 49 states in conducting CWD testing on free-ranging cervids. Methods and sampling levels vary; however, most other states also test some combination of roadkill, hunter-harvested deer, and deer exhibiting clinical symptoms of disease. Forty-five states in addition to Texas also test captive cervids for CWD, although in some states this testing is voluntary. The four states that don’t test for CWD in captive cervids—Nevada, South Carolina, Washington, and Wyoming—either don’t permit captive cervids or have a nominal number of ranches with captive animals.
Bans on the importation and movement of cervid carcasses and body parts also are common—41 states join Texas in implementing restrictions or outright bans but with exceptions for items such as deboned meat, cleaned hides, and taxidermy mounts. States including Arkansas, Michigan, Minnesota, Virginia, and West Virginia have implemented CWD management or containment zones with enhanced movement restrictions and testing.
CHRONIC WASTING DISEASE FUNDING
TPWD reports CWD-related expenditures pursuant to the General Appropriations Act (GAA), Article VI, Parks and Wildlife Department, Strategy A.1.1, Wildlife Conservation. The Legislature appropriated $147.6 million in All Funds to TPWD from fiscal years 2011 to 2017 within this strategy, which includes funding for the regulation and management of other species of animals, management and operation of TPWD’s wildlife management areas, wildlife surveys and research, and the issuance of wildlife permits. According to TPWD, CWD- related expenditures for the same period totaled approximately $4.5 million (3.1 percent of appropriations within the Wildlife Conservation strategy), and other expenditures related to general disease management totaled $64,880 during the period. As Figure 3 shows, the majority of the CWD-related expenditures are financed by Federal Funds. TPWD reports that these funds consist of the following grants: (1) a federal grant for CWD surveillance issued as part of a cooperative agreement between TPWD and the USDA Animal and Plant Health Inspection Service; and (2) a portion of a Federal Aid in Wildlife Restoration Act grant that was used to carry out CWD monitoring and
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
$1,800,000.0 $1,600,000.0 $1,400,000.0 $1,200,000.0 $1,000,000.0 $800,000.0 $600,000.0 $400,000.0 $200,000.0 $0.0 License Plate Trust Fund 2012 Appropriated Receipts 2011 2013 2014 2015 Federal Funds 2016 2017 General Revenue–Dedicated Funds Account No. 9, Game, Fish, and Water Safety
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OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
testing. The Federal Aid in Wildlife Restoration Act, commonly known as the Pittman-Robertson Act, provides cost-shared federal aid to states on a formula basis for the management and restoration of wildlife.
Expenditures increased during fiscal years 2016 and 2017 due to expanded TPWD activities involving CWD following the detection of the disease in a deer breeding facility in June 2015, as discussed in the following section.
Figure 4 shows full-time-equivalent positions for hours that TPWD staff attributed to CWD-related tasks for fiscal years 2011 to 2017.
TAHC receives appropriations for a CWD program pursuant to GAA, Article VI, Animal Health Commission, Strategy A.1.1, Field Operations, which covers the agency’s statewide, field-based, animal health management and assurance programs. The Legislature initiated the CWD program funding during fiscal year 2005 for the purpose of furthering CWD surveillance in breeder deer and in elk, decreasing the risk of introduction of CWD, and providing early disease detection. TAHC also uses appropriations outside the CWD program for purposes related to CWD. As shown in Figure 5, total CWD-related expenditures for the agency from fiscal years 2005 to 2017 are approximately $2.1 million in General Revenue Funds, or 1.2 percent of total agency appropriations during that period (approximately $171.9 million in All Funds).
FIGURE 4
TEXAS PARKS AND WILDLIFE DEPARTMENT FULL-TIME- EQUIVALENT POSITIONS RELATED TO CHRONIC WASTING DISEASE
FISCAL YEARS 2011 TO 2017
3.03 2.98 3.08 1.24 14.44 12.05 3.95
2011 2012 2013 2014 2015 2016 2017
Note: Texas Parks and Wildlife staff hours shown are attributed to chronic wasting disease-related tasks in terms of full-time- equivalent positions.
Source: Texas Parks and Wildlife Department.
TEXAS REGULATORY RESPONSE TO CHRONIC WASTING DISEASE
The state’s regulatory response to CWD began in calendar year 1999 with TAHC’s development of a voluntary status- monitoring program in which participating owners of herds were required to submit annual inventories and submission of samples from all cases of mortality in animals age 17 months or older. In 2002, following the discovery of CWD in multiple other states, TPWD began testing roadkill and
FIGURE 5
TEXAS ANIMAL HEALTH COMMISSION CHRONIC WASTING DISEASE-RELATED EXPENDITURES FISCAL YEARS 2005 TO 2017
$600,000.0 $500,000.0 $400,000.0 $300,000.0 $200,000.0 $100,000.0 $0.0 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017
Source: Texas Animal Health Commission.
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FIGURE 6
CHRONIC WASTING DISEASE EVENTS IN TEXAS CALENDAR YEARS 2012 TO 2017
a an Note: CWD=chronic wasting disease; TAHC=Texas Animal Health Commission; TPWD=Texas Parks and Wildlife Department; TTT=Trap, Transport, and Transplant Permit Program; DMP=Deer Management Permit. Source: Legislative Budget Board.
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
CWD confirmed in free-ranging mule deer TAHC adopts rules establishing CWD containment zones [42]
CWD confirmed in captive white-tailed deer
TPWD adopts emergency CWD rules for deer breeder permits
TPWD adopts interim CWD rules for DMPs
CWD confirmed in free-ranging white-tailed deer 2012
2016 2017 2018 establishing CWD TPWD adopts emergency CWD rules 2013 2014 2015
ding surveillance of and movement restrictions for CWD-susceptible exotic livestock containment zones
TAHC adopts rules expanding surveillance of and movement restrictions for CWD-susceptible exotic livestock for TTT permits and DMPs
TPWD adopts interim CWD rules for deer breeder permits
TPWD adopts comprehensive CWD rules
TPWD convenes facilitated negotiation process for comprehensive CWD rules
hunter-harvested deer for CWD, and TPWD and TAHC adopted rules intended to prevent the importation of potentially diseased deer and elk into the state. Testing by TPWD and rulemaking from both agencies continued until 2005, when, at the recommendation of TPWD’s White-tailed Deer Advisory Committee, TPWD closed the Texas border to the entry of out-of-state captive white- tailed and mule deer and increased regulatory requirements regarding disease monitoring and record keeping due to the threat that CWD posed. Those rules were updated in 2010 to address other disease threats to white-tailed and mule deer.
Despite these efforts, Texas confirmed the first state cases of CWD in July 2012 among free-ranging mule deer in Hudspeth County, part of a western region of the state known as the Trans-Pecos. Figure 6 shows a timeline of significant events related to the discovery of CWD in Texas, beginning with these confirmed cases in 2012. TPWD, TAHC, and the CWD Task Force already had collaborated on a CWD management plan with a response structured for the region after the detection of the disease in mule deer harvested in New Mexico within two miles of the Texas border. The plan called for the establishment of movement restriction zones in the region. The zones were established by TAHC rule in September 2012 and TPWD rule in November 2012. TAHC also adopted rules in June 2013 expanding its surveillance of elk, which began in December 2005, to include other CWD-susceptible exotic livestock.
CWD cases were confined to Hudspeth County until June 2015, when the disease was confirmed in a captive white- tailed deer in a Medina County deer breeding facility west of San Antonio. The Medina County tissue samples were submitted by the breeder facility as part of routine deer mortality surveillance. TPWD responded by temporarily disabling access to the online database by which deer breeders obtain transfer permits to transport deer, placing movement restrictions on breeder facilities that had received deer from the Medina County facility or shipped deer to the facility during the previous two years, and disallowing release of captive deer from all breeder facilities into the wild.
TPWD adopted emergency rules in August 2015 that included the following requirements: (1) specific testing requirements for deer breeders to move deer to other deer breeders or for purposes of release; (2) similar testing requirements on release sites; and (3) restriction of the release of breeder deer to enclosures surrounded by a fence of at least seven feet in height capable of retaining deer at all times. TPWD also adopted emergency rules in October 2015 to address movement of white-tailed or mule deer in accordance
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OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
FIGURE 7
AFFILIATIONS OF PARTICIPANTS IN FACILITATED NEGOTIATIONS WITH CHRONIC WASTING DISEASE STAKEHOLDERS FEBRUARY 2016 TO APRIL 2016
2015 Stakeholder Group
Breeder User Group
Chronic Wasting Disease Task Force
Deer Breeders Corporation
Exotic Wildlife Association
North American Deer and Elk Farmers Association Source: Texas Parks and Wildlife Department.
Private Lands Advisory Committee Texas Deer Association
Texas Parks and Wildlife Department Texas Wildlife Association White-tailed Deer Advisory Committee Deer Breeders Corporation
with a trap, transport, and transplant (TTT) permit or a deer management permit (DMP).
TPWD replaced the emergency rules with interim rules in November 2015 for deer breeders and in January 2016 for DMP (the TTT emergency rules were allowed to expire). The agency intended for the interim rules to maintain regulatory continuity during the 2015–2016 deer season and the period immediately thereafter, and to review all the interim rules following the close of the season.
That review began in February 2016, when TPWD invited a group of stakeholders, shown in Figure 7, to participate in a negotiation process facilitated by the Center for Public Policy Dispute Resolution at the University of Texas School of Law. According to TPWD, the agency took this step to address criticisms from some deer breeders that official and ad hoc TPWD advisory committees were “stacked” with members predisposed against the interests of the deer- breeding industry. The purpose of the negotiation was to develop a consensus concerning the essential components of eventual regulations to comprehensively address and implement effective CWD management strategies. Stakeholders represented various interests, including deer breeders, landowners, hunters, veterinarians, wildlife enthusiasts, TAHC, and TPWD. The results of the negotiation formed the basis of comprehensive rules, which were proposed in April 2016 and adopted in June 2016, following the solicitation of public comment and public testimony. Those rules remain in place as of October 2018, with some modification, such as the adjustment of the CWD movement restriction zones set by TPWD and TAHC.
The detection of CWD continues in Texas, with most cases found in white-tailed breeder deer from one of five deer- breeding facilities, and the most recent cases confirmed in December 2018, as of January 2019. The total for calendar years 2012 to 2018 is 139 out of approximately 131,000 tests conducted beginning in fiscal year 2003. Figure 8 shows the number of positive CWD cases in Texas from calendar years 2012 to 2018.
In comparison, Wisconsin, which has had a widely studied CWD outbreak since 2002, has recorded more than 4,200 positive CWD cases in free-ranging and captive deer from more than 210,000 tested samples. Wisconsin’s population of white-tailed deer was estimated at approximately 1.4 million in 2017.
To test white-tailed and mule deer in Texas, TPWD pays for the costs of general CWD surveillance, and the holders of TPWD deer-related permits and the owners of deer- release sites pay for testing related to permitted activities. To test nonnative cervids, landowners pay for the testing of the first three cervids harvested on their properties to comply with TAHC’s CWD surveillance requirements. Testing costs vary depending on the type of sample and how samples are collected, but costs are a minimum $25 for a single tissue test for both TPWD and private individuals. Testing levels are based on herd-level statistical sampling where the number of samples can decrease for bigger herds without sacrificing statistical confidence in detecting the disease.
DEER-BREEDING INDUSTRY RESPONSE TO REGULATION
Representatives of the deer-breeding industry have been vocal critics of the state’s regulatory response to CWD. The rules adopted following the discovery of CWD in captive white-tailed deer require deer-breeding facilities to meet certain testing standards for deer to be moved under TPWD transfer permits. In addition to resulting in an administrative burden to meet those standards, the rules limit the ability of some facilities to transfer deer for sale or purchase. This limitation has led some deer breeders to claim that they are being singled out unfairly by regulators. TPWD’s response
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FIGURE 8
POSITIVE CHRONIC WASTING DISEASE CASES IN TEXAS CALENDAR YEARS 2012 TO 2018 Source: Texas Parks and Wildlife Department.
centers on the agency’s determination that more than 75.0 percent of the deer breeders in Texas were linked by no more than three degrees of separation to the facility in Medina County where the first CWD-positive white-tailed deer was discovered. This scale of interconnectedness, coupled with the risk of inadvertently moving CWD to new areas of the state posed by the artificial movement of deer (e.g., in a trailer as part of a transfer between deer breeders), informed TPWD’s imposition of restrictions on movement. However, the privilege of movement enables deer breeders to set a market outside of paid access to deer on a breeder’s own property.
A second element of criticism involves the perception that regulators are acting on a stigma against breeder deer. Some critics outside the deer-breeding industry argue that breeder deer are unnatural, more prone to diseases such as CWD due to the circumstances of their captivity, and more likely to spread disease as part of the industry. Respondents to that criticism have attributed this perception to jealousy of the trophies that deer breeders are able to raise or fear of the industry’s encroachment on hunting operations that tout the quality of free-ranging deer. This notion of a stigma also is related to the issue of deer tagging or identification. The Texas Parks and Wildlife Code requires breeder deer to be identified by a visible identification tag while held in a permitted deer-breeding facility. Recent efforts to expand the means of identification to include scannable subcutaneous microchips, which are nonvisible and supported by some deer breeders, have been resisted by some ranchers and hunters who state that visible identification is necessary to track and contain potentially diseased breeder deer that could threaten free-ranging deer’s health.
Another aspect of criticism centers on the issue of private ownership. Some deer breeders state that captive deer are privately owned and, therefore, outside of TPWD’s regulatory jurisdiction. TPWD holds that deer breeders possess deer in bailment, or without the rights of ownership, because state law deems that all wild animals inside state borders are the property of the people of the state. According to TPWD, breeder deer are never sold in the legal sense; a deer breeder receives monetary compensation for transferring the permitted privilege of possession of a breeder deer to another permitted deer breeder or for agreeing to release a breeder deer on a landowner’s property.
As shown in Figure 9, the number of permitted deer breeders in Texas decreased during permit years 2016 and 2017, and TPWD projects that trend will continue for permit year 2018. (A permit year begins July 1 and ends June 30 of the following calendar year.) Certain TPWD
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
60 50 40 30 20 10 0 2012 2013 2014 2015 2016 2017 2018 Panhandle Mule Deer – Free Range Panhandle White-tailed Deer – Free Range South-Central Texas White-tailed Deer – Breeder Pen South-Central Texas Elk – Breeder Release Site Trans Pecos Mule Deer – Free Range Panhandle Elk – Free Range South-Central Texas White-tailed Deer – Free Range South-Central Texas White-tailed Deer – Breeder Release Site LEGISLATIVE BUDGET BOARD STAFF – APRIL 2019 LEGISLATIVE BUDGET BOARD STAFF REPORTS – ID: 4830 9
OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE
testing requirements for compliant deer-release sites will expire March 1, 2019, because the testing regimen within that period will have produced statistical confidence that enables the expiration. Legislative Budget Board staff found no indications that the collaboration between TAHC and TPWD results in duplication of effort, nor that either agency exceeds its scope of authority or fails to engage stakeholders adequately in response to CWD.
FIGURE 9
TEXAS PARKS AND WILDLIFE DEPARTMENT DEER BREEDER PERMITS ISSUED, PERMIT YEARS 2002 TO 2018
1400 1300 1200 1100 1000 900 800 700 600 500 2002 2004 2006 2008 2010 2012 2014 2016 2018
Note: Amount for permit year 2018 is projected. Source: Texas Parks and Wildlife Department.
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TEXAS LEGISLATORS AND HOW TO LEGISLATE CWD TSE PRION DISEASE
TEXAS Legislators need to crawl out of bed with the damn captive deer farmers and start closing them down if they cannot keep them from spreading cwd to hell and back. that's what you need to tell the Texas Legislation...
just my opinion...kind regards, terry
TEXAS CHRONIC WASTING DISEASE CWD TSE PRION TOTALS MOVE UP TO 449 TO DATE CWD Positives in Texas
CWD Positive
Confirmation Date Free Range/Captive County Source Species Sex Age
Pending Breeder Deer Hunt Facility #9 White-tailed Deer F 2.5
Pending Breeder Deer Hunt Facility #9 White-tailed Deer M 2.6
Pending Breeder Deer Hunt Facility #9 White-tailed Deer M 2.5
Pending Breeder Deer Hunt Facility #9 White-tailed Deer F 2.5
2023-01-03 Free Range Hartley N/A Mule Deer M 4.5
2023-01-03 Free Range Hartley N/A Mule Deer M 4.5
2023-01-03 Free Range Hartley N/A Mule Deer M 4.5
2023-01-03 Free Range Hartley N/A Mule Deer M 4.5
2023-01-03 Free Range Dallam N/A Mule Deer M 1.5
2023-01-03 Free Range Hartley N/A Mule Deer M 4.5
2023-01-03 Free Range Hartley N/A Mule Deer M 5.5
2023-01-03 Breeder Deer Hunt Facility #9 White-tailed Deer M 2.4
2022-12-30 Free Range Medina N/A White-tailed Deer M 4.5
2022-12-30 Breeder Deer Hunt Facility #9 White-tailed Deer M 2.5
2022-12-30 Breeder Release Site Uvalde Facility #3 White-tailed Deer Unknown Unknown
2022-12-22 Breeder Deer Hunt Facility #9 White-tailed Deer M 4.5
2022-12-22 Breeder Deer Hunt Facility #9 White-tailed Deer F 4.5
2022-12-22 Breeder Deer Hunt Facility #9 White-tailed Deer M 4.5
2022-12-16 Breeder Deer Hunt Facility #9 White-tailed Deer F 4.5
2022-12-16 Breeder Release Site Uvalde Facility #3 White-tailed Deer Unknown 6.0
2022-12-16 Breeder Deer Hunt Facility #9 White-tailed Deer M 2.5
2022-12-15 Breeder Release Site Medina N/A White-tailed Deer F 6.5
2022-12-15 Breeder Release Site Uvalde Facility #3 White-tailed Deer Unknown 5.0
2022-12-15 Breeder Deer Hunt Facility #9 White-tailed Deer M 2.5
Showing 1 to 24 of 449 entries
(***> TO SEE A BETTER PICTURE OF WILD CWD VS CAPTIVE CWD, EXPAND THE PAGE...tss <***)
CHRONIC WASTING DISEASE CASESCWD STATUS OF CAPTIVE HERDS Updated January 2023
Date of Index Case Confirmation Index Case State County Species Herd Type HCP Enrolled HCP Certified Number of Animals Herd Status
/13/2022 3 YR Female TX Limestone WTD Breeder Yes Yes 526 Quarantine
8/30/2022 1 YR Male TX Gillespie WTD Breeder No No 74 Quarantine
8/24/2021 3Y Female TX Duval WTD Breeder No No 188 Depopulated
6/15/2021 4 Y Female TX Uvalde WTD Breeder & Shooter No No 1000+ Quarantine
4/20/2021 1.5 Y Male TX Mason WTD Breeder Traceback Yes Yes 93 Depopulated
4/20/2021 1.5 Y Male TX Matagorda WTD Breeder Traceback Yes No 221 Depopulated
3/30/2021 3.5 Y, 2.5 Y, 3.5Y TX Uvalde WTD Breeder Yes Yes 61 Depopulated
3/30/2021 2.5 Y & 1.5 Y TX Uvalde WTD Breeder Yes No 318 Depopulated
3/29/2021 3Y Female TX Hunt WTD Breeder Yes No 381 Quarantine
2/2020 5.5 Y Female TX Kimball WTD Breeder No NA 80 Depopulated
7/2017 4Y Male TX Medina WTD Breeder No NA 98 Depopulated
TAHC CWD Summary Minutes of the 414th Commission Meeting November 15, 2022
Summary Minutes of the 414th Commission Meeting – 11/15/2022
snip...
• Chronic Wasting Disease (CWD):
2021 Incident
310 total affected herds
o Seven positive facilities: three facilities in Uvalde, one in Duval, one in Hunt, one in Matagorda, & one in Mason
o 303 Trace herds:
177 Breeder facilities, 117 release sites, four nursing facilities, five DMP sites
o Status:
Released after meeting requirements:
- 164 breeder facilities, 58 release sites/DMPs/nursing facilities
Summary Minutes of the 414th Commission Meeting – 11/15/2022
3
Under a herd plan: three breeder facilities, 23 release sites/DMP
Pending a signed herd plan: three breeder facilities, 44 release sites/DMP
Out of state: eight facilities
2022 Incident
84 total affected herds
o 2 Positive facilities:
Gillespie County – Confirmed August 30th
Limestone County – Confirmed
o 82 Trace facilities: 22 breeder sites, 59 release sites, and one DMP site
o Status
Released after meeting requirements: seven breeder facilities, 2 release sites
Signed herd plan: nine release sites
Pending a signed herd plan: 15 Breeder facilities, 49 Release sites/DMP
USDA VS review of the Texas CWD HCP UPDATE
o TAHC is working with TPWD to modify the TWIMS database to better support the HCP
o Review of all enrolled herds has begun, and is to be completed by the end of February 2023
snip...
Cooperative Award Award End Budget Rcd $$ Spent Encumbered Need to Spend
21 CWD Depop/Genome Study 9/14/2022 $429,598 $375,032 $375,032 $0 $56,566
21 CWD D4 TX (Depop) 9/14/2022 $274,968 $274,968 $274,968 $0 $0
22 CWD Lng Rnge/Low Energy 8/31/2023 $250,000 $0 $0 $0 $250,000
FY 2022-2023 Collected Fee Revenue:
$9,500 from CWD Inspection was projected, $16,550 was received year to date
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Item 13 – Consideration of and Possible Action on Proposed Rules Mr. Jabbar Fahim discussed the following Regulation Proposals:
snip...
b) Chapter 40, Chronic Wasting Disease
The Texas Animal Health Commission (commission) proposes amendments to Title 4, Texas Administrative Code, Chapter 40, Chronic Wasting Disease. The proposed amendments both increase surveillance and reduce the risk of chronic wasting disease (CWD) being spread from areas where it may exist. §40.6 proposed adoption establishes one new containment zone (CZ) 5, expands existing CZ 2 and CZ 3, creates a new surveillance zone (SZ) 8, and modifies existing SZ 5 to either implement or improve surveillance efforts as part of the agency’s effort to manage chronic wasting disease (CWD).
snip...
Counties where CWD Exposed Deer were Released
Number of CWD Exposed Deer Released by County
“Regarding the current situation involving CWD in permitted deer breeding facilities, TPWD records indicate that within the last five years, the seven CWD-positive facilities transferred a total of 2,530 deer to 270 locations in 102 counties and eight locations in Mexico (the destinations included 139 deer breeding facilities, 118 release sites, five Deer Management Permit sites, and three nursing facilities).'' ...
It is apparent that prior to the recent emergency rules, the CWD detection rules were ineffective at detecting CWD earlier in the deer breeding facilities where it was eventually discovered and had been present for some time; this creates additional concern regarding adequate mitigation of the risk of transferring CWD-positive breeder deer to release sites where released breeder deer come into contact with free-ranging deer...
Commission Agenda Item No. 5 Exhibit B
DISEASE DETECTION AND RESPONSE RULES
PROPOSAL PREAMBLE
1. Introduction.
snip...
A third issue is the accuracy of mortality reporting. Department records indicate that for each of the last five years an average of 26 deer breeders have reported a shared total of 159 escapes. Department records for the same time period indicate an average of 31 breeding facilities reported a shared total of 825 missing deer (deer that department records indicate should be present in the facility, but cannot be located or verified).
TEXAS CWD STRAIN
“Wow,” he said. “Unlike anything we've seen before.”
The prions from the Texas deer were a lot harder to destroy than the ones from the Colorado elk. In fact, the guanidine barely damaged them at all. “We’ve never seen that before in any prion strain, which means that it has a completely different structure than we've ever seen before,” says Zabel. And that suggests that it might be a very different kind of chronic wasting disease. The researchers ran the same test on another Texas deer, with the same results.
TEXAS CWD STRAIN
77. Assessing chronic wasting disease strain differences in free-ranging cervids across the United States
Kaitlyn M. Wagnera, Caitlin Ott-Connb, Kelly Strakab, Bob Dittmarc, Jasmine Battend, Robyn Piercea, Mercedes Hennessya, Elizabeth Gordona, Brett Israela, Jenn Ballarde and Mark D Zabela
aPrion Research Center at Colorado State University; bMichigan Department of Natural Resources; cTexas Parks and Wildlife Department; dMissouri Department of Conservation, 5. Arkansas Game and Fish Commission CONTACT Kaitlyn M. Wagner miedkait@rams.colostate.edu
ABSTRACT
Background/Introduction: Chronic wasting disease (CWD) is an invariably fatal prion disease affecting captive and free-ranging cervids, including white-tailed deer, mule deer, moose, elk, and reindeer. Since the initial description of the disease in the 1960’s, CWD has spread to 23 states, 3 Canadian Provinces, South Korea, Norway and, most recently, Finland. While some outbreaks of CWD were caused by transport of infected animals from endemic regions, the origin of CWD in other epizootics is unclear and has not been characterized. Previous studies have shown that there are two distinct strains of CWD. However, the continuous spread and the unclear origin of several outbreaks warrant continued surveillance and further characterization of strain diversity.
Materials and Methods: To address these knowledge gaps, we used biochemical tests to assess strain differences between CWD outbreaks in Michigan, Texas, Missouri, and Colorado, USA. Brain or lymph node samples were homogenized and digested in 50 µg/mL proteinase K (PK). These samples were then run on a Western blot to assess glycoform ratio and electrophoretic mobility. Texas samples were digested in 100 µg/mL PK. To assess conformational stability, brain or lymph node homogenates were incubated in increasing concentrations of guanidine hydrochloride from 0 M to 4 M in 0.5 M increments. Samples were then precipitated in methanol overnight, washed and PK digested in 50 µg/mL PK before slot blotting.
Results: Our results have found significant differences in glycoform ratio between CWD from Michigan and Colorado, but no differences were observed in conformational stability assays. Interestingly, when testing our CWD isolates from Texas to analyse electrophoretic mobility and glycoform ratio, we found that these samples did not exhibit the characteristic band shift when treated with PK, but PK resistant material remained. Additionally, results from our conformational stability assay demonstrate a unique profile of these Texas isolates. Testing of samples from Missouri is currently underway.
Conclusions: Thus far, our data indicate that there are strain differences between CWD circulating in Michigan and CWD in Colorado and provide important insight into CWD strain differences between two non-contiguous outbreaks. We have also identified a unique strain of CWD in Texas with biochemical strain properties not seen in any of our other CWD isolates. These results highlight the importance of continued surveillance to better understand this devastating disease. These results have important implications for CWD emergence, evolution and our understanding of prion strain heterogeneity on the landscape.
The disease devastating deer herds may also threaten human health
Scientists are exploring the origins of chronic wasting disease before it becomes truly catastrophic.
Rae Ellen Bichell
Image credit: David Parsons/Istock
April 8, 2019
This story was published in collaboration with the Mountain West News Bureau, a collaboration between Wyoming Public Media, Boise State Public Radio in Idaho, KUER in Salt Lake City and KRCC and KUNC in Colorado.
SNIP...
One day in late February, in their laboratory in Fort Collins, Colorado, Wagner and Zabel compared the prions from the brains of CWD-infected deer in Texas with those of elk in Colorado. They want to know if the proteins were all mangled in the same way, or not. “If they are different, this would suggest that we have different strain properties, which is evidence as we're building our case that we might have multiple strains of CWD circulating in the U.S.,” says Wagner.
Step one is to see if they’re equally easy to destroy using a chemical called guanidine. The shape of a prion dictates everything, including the way it interacts with an animal’s cells and the ease with which chemicals can unfold it.
“Moment of truth,” said Wagner, as she and Zabel huddled around a computer, waiting for results to come through. When they did, Zabel was surprised.
“Wow,” he said. “Unlike anything we've seen before.”
The prions from the Texas deer were a lot harder to destroy than the ones from the Colorado elk. In fact, the guanidine barely damaged them at all. “We’ve never seen that before in any prion strain, which means that it has a completely different structure than we've ever seen before,” says Zabel. And that suggests that it might be a very different kind of chronic wasting disease. The researchers ran the same test on another Texas deer, with the same results.
Now, these are only the preliminary results from a few animals. Wagner and Zabel have a lot more experiments to do. But if future tests come to the same conclusion, it would support their hypothesis that there are multiple strains of chronic wasting disease out there, all with different origins. That, in turn, could mean that this disease will become even trickier to manage than it already is.
And, Zabel adds, there’s something else. “If it's still evolving, it may still evolve into a form that could potentially, eventually affect humans,” he says.
Zabel is not the only one worried about that possibility.
OSTERHOLM, THE EPIDEMIOLOGIST from Minnesota, is also concerned. He directs the Center for Infectious Disease Research and Policy at the University of Minnesota, and is serving a one-year stint as a “Science Envoy for Health Security” with the U.S. State Department. In February, he told Minnesota lawmakers that when it comes to chronic wasting disease, we are playing with fire. “You are going to hear from people that this is not going to be a problem other than a game farm issue. You're going to hear from people that it's not going to transmit to people, and I hope they're right, but I wouldn't bet on it,” he said. “And if we lose this one and haven’t done all we can do, we will pay a price.”
If that wasn’t warning enough, he added: “Just remember what happened in England.”
He was talking about mad cow disease. Decades ago, Osterholm got involved in studying the potential for the newly emerging condition — bovine spongiform encephalopathy, or BSE for short — to be transmitted to humans.
At that point, researchers had yet to document a prion disease in animals that could infect people. They did, however, have a few pieces of the puzzle. For one, work in Papua New Guinea had shown that people could transmit prion diseases to each other if they practiced cannibalism, especially of the brain-eating variety. They also knew that BSE was spreading quickly between cattle. Osterholm says he and others worried that the more widespread it became, the more chances it might have to change into something that could sicken people.
“A lot of people thought that it was an overreaction,” says Osterholm. “Then, of course, in 1996, 10 years later, we recognized that in fact transmission had occurred.” Variant Creutzfeldt-Jakob disease, as the illness is called when it appears in human beings, has infected about 230 people worldwide. Osterholm says he feels like he’s having déjà vu, except that instead of mad cow, now it’s chronic wasting disease that’s spreading in animals, with the potential to cross the species barrier to infect humans.
SNIP...SEE FULL TEXT;
***> TEXAS BREEDER DEER ESCAPEE WITH CWD IN THE WILD, or so the genetics would show?
OH NO, please tell me i heard this wrong, a potential Texas captive escapee with cwd in the wild, in an area with positive captive cwd herd?
apparently, no ID though. tell me it ain't so please...
23:00 minute mark
''Free Ranging Deer, Dr. Deyoung looked at Genetics of this free ranging deer and what he found was, that the genetics on this deer were more similar to captive deer, than the free ranging population, but he did not see a significant connection to any one captive facility that he analyzed, so we believe, Ahhhhhh, this animal had some captive ahhh, whatnot.''
Biologists Stress Testing Harvests for Chronic Wasting Disease During Deer Season
Dec. 2, 2022
Media Contact: TPWD News, Business Hours, 512-389-8030
April 22, 2016
Scrapie Confirmed in a Hartley County Sheep
AUSTIN - Texas Animal Health Commission (TAHC) officials have confirmed scrapie in a Hartley County ewe. The ewe was tested by TAHC after the owner reported signs of weight loss and lack of coordination to their local veterinarian. The premises was quarantined and a flock plan for monitoring is being developed by the TAHC and USDA.
"The TAHC is working closely with the flock owner, sharing all of the options for disease eradication," said Dr. David Finch, TAHC Region 1 Director. "We are thankful the producer was proactive in identifying a problem and seeking veterinary help immediately."
Texas leads the nation in sheep and goat production. Since 2008, there have been no confirmed cases of scrapie in Texas. The last big spike in Texas scrapie cases was in 2006 when nine infected herds were identified and the last herd was released from restrictions in 2013.
According to USDA regulations, Texas must conduct adequate scrapie surveillance by collecting a minimum of 598 sheep samples annually. Since USDA slaughter surveillance started in FY 2003, the percent of cull sheep found positive for scrapieat slaughter (once adjusted for face color) has decreased 90 percent.
Scrapie is the oldest known transmissible spongiform encephalopathies, and under natural conditions only sheep and goats are known to be affected by scrapie. It is a fatal disease that affects the central nervous system of sheep and goats. It is not completely understood how scrapie is passed from one animal to the next and apparently healthy sheep infected with scrapie can spread the disease. Sheep and goats are typically infected as young lambs or kids, though adult sheep and goats can become infected.
The most effective method of scrapie prevention is to maintain a closed flock. Raising replacement ewes, purchasing genetically resistant rams and ewes,or buying from a certified-free scrapie flock are other options to reduce the risk of scrapie. At this time the resistant genetic markers in goats have not been identified, therefore it is important to maintain your sheep and goat herds separately.
The incubation period for Scrapie is typically two to five years. Producers should record individual identification numbers and the seller's premise identification number on purchase and sales records. These records must be maintained for a minimum of five years.
Producers should notify the Texas Animal Health Commission (800-550-8242) or the USDA-Austin Office (512-383-2400) if they have an adult sheep or goat with neurologic signs such as incoordination, behavioral changes, or intense itching with wool loss. Producers may order scrapie identification tags by calling 866-873-2824. For more information, please visit our website at:
web.archive.org/web/20160607024701/http://www.tahc.texas.gov/news/pr/2016/2016-04-22_TAHCScrapie.pdf
Scrapie: The flock identified in April 2016 remains under quarantine in Hartley County.
Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie TSE Prion Experiment 1964 How Did CWD Get Way Down In Medina County, Texas?
DISCUSSION
Observations of natural outbreaks of scrapie indicated that the disease spread from flock to flock by the movement of infected, but apparently normal, sheep which were incubating the disease.
There was no evidence that the disease spread to adjacent flocks in the absent of such movements or that vectors or other host species were involved in the spread of scrapie to sheep or goats; however, these possibilities should be kept open...
snip...PLEASE READ THE FULL REPORT ON THE SCRAPIE FIELD TRIAL EXPERIMENTS IN MISSION, TEXAS!!!
TEXAS CWD 429 CASES CONFIRMED TO DATE
Texas Chronic Wasting Disease CWD TSE Prion Increases To 428 Confirmed To Date
Biologists Stress Testing Harvests for Chronic Wasting Disease During Deer Season
DEC. 2, 2022
MEDIA CONTACT: TPWD NEWS, BUSINESS HOURS, 512-389-8030
News Image Share on Facebook Share Release URL
AUSTIN— With the recent discovery of Chronic Wasting Disease (CWD) in Kaufman County, Texas Parks and Wildlife Department (TPWD) biologists are stressing the importance of testing harvested deer taken this hunting season to curb the spread of this deadly disease.
CWD is a highly contagious and fatal neurological disease affecting members of the deer family such as white-tailed deer, mule deer, elk and moose. Hunters and landowners can voluntarily submit their harvest for free CWD testing at a TPWD check station or by contacting local wildlife biologists.
First recognized in 1967 in captive mule deer in Colorado, CWD has since been documented in captive and/or free-ranging deer in 30 states and three Canadian provinces. To date, 428 captive or free-ranging cervids — including white-tailed deer, mule deer, red deer, and elk — in 17 Texas counties have tested positive for CWD.
Eradication of CWD is very difficult if not impossible when established in free-ranging deer populations and in the environment. Testing for CWD allows wildlife biologists and animal health officials to get a clearer picture of the prevalence and distribution of the disease across Texas. Proactive monitoring improves the state’s response time to a CWD detection and can greatly reduce the risk of the disease further spreading to neighboring captive and free-ranging populations.
Those hunting in CWD surveillance and containment zones need to know the submission requirements
for susceptible species before hitting the field this season. Anyone hunting in an established CWD zone must bring their deer to a check station within 48 hours of harvest for testing.
There are about 30 check stations and drop boxes across the state in Kimble County, far West Texas, South Central Texas, the northwest Panhandle, Val Verde County, Hunt County, Lubbock County, Gillespie County, Limestone County and Duval County.
For more information about CWD, visit the TPWD website, the Texas Animal Health Commission website, or read about how hunters can help protect deer from CWD with a story from the Texas Parks and Wildlife Magazine.
Find TPWD veterinarians speaking about CWD on the TPWD YouTube page.
Texas CWD Confirmed High Fence Release Site Kaufman County, To Date 420 Cases Confirmed
Chronic Wasting Disease Detected at a High Fence Release Site in Kaufman County
Nov. 28, 2022 Media Contact: TPWD News, Business Hours, 512-389-8030
AUSTIN –Chronic Wasting Disease (CWD) was detected on a high fence release site in Kaufman County. This is the first positive detection of CWD in the county.
The white-tailed buck, harvested at a high-fence release site located in a CWD Surveillance Zone, was delivered to a Hunt County CWD check station in compliance with surveillance zone requirements. Texas Parks and Wildlife Department (TPWD) and Texas Animal Health Commission (TAHC) received notice of the CWD-positive test result from the Texas A&M Veterinary Medical Diagnostic Laboratory (TVMDL) on Nov. 14.
Prior to this detection, the high-fence release site was identified as a “trace herd” property to the Hunt County CWD-positive deer breeding facility reported on March 31, 2021, meaning deer were transferred from the Hunt County facility to this release site prior to discovery of CWD in that facility. Plans to conduct additional CWD investigations are underway.
“The incubation period of CWD can span years, creating disease detection and management challenges as seen in this recent detection,” said Andy Schwartz, TAHC Executive Director and State Veterinarian.
Animal health and wildlife officials will continue investigations to determine the extent of the disease within the property and mitigate risks to Texas’ CWD-susceptible species. Adequate surveillance and quick detection of CWD can help mitigate the disease’s spread.
“The discovery of CWD on this ranch is an unfortunate situation that TPWD and TAHC take very seriously,” said John Silovsky, TPWD Wildlife Division Director. “Both agencies will respond appropriately to this matter to protect the state’s susceptible species from further disease exposure. Hunters are reminded of the requirement to bring their harvested deer to the check station within 48 hours of harvest.”
First recognized in 1967 in captive mule deer in Colorado, CWD has since been documented in captive and/or free-ranging deer in 30 states and three Canadian provinces. To date, 420 captive or free-ranging cervids — including white-tailed deer, mule deer, red deer, and elk — in 17 Texas counties have tested positive for CWD. For more information on previous detections visit the CWD page on the TPWD website.
Testing for CWD allows wildlife biologists and animal health officials to get a clearer picture of the prevalence and distribution of the disease across Texas. Proactive monitoring improves the state’s response time to a CWD detection and can greatly reduce the risk of the disease further spreading to neighboring captive and free-ranging populations.
This confirmation is a good reminder to those hunting in CWD surveillance and containment zones to know the submission requirements for CWD susceptible species. Additionally, hunters outside of established surveillance and containment zones are encouraged to voluntarily submit their harvest for testing at a check station, for free, before heading home from the field. A map of TPWD check stations for all CWD zones can be found on the TPWD website.
For more information about CWD, visit the TPWD web site or the TAHC web site.
Counties where CWD Exposed Deer were Released
Number of CWD Exposed Deer Released by County
Texas Chronic Wasting Disease Discovered at a Deer Breeding Facility in Gillespie County
Media Contacts: TAHC Communications public_info@tahc.texas.gov, 512.719.0750 TPWD Press Office news@tpwd.texas.gov, 512.389.8030
For Immediate Release
September 2, 2022
Chronic Wasting Disease Discovered at a Deer Breeding Facility in Gillespie County
AUSTIN, TX – Chronic Wasting Disease (CWD) has been discovered in a deer breeding facility in Gillespie County. The Texas Parks and Wildlife Department (TPWD) and Texas Animal Health Commission (TAHC) are collaboratively working to determine the source and extent of the first positive detection of the disease in this county.
snip...
First recognized in 1967 in captive mule deer in Colorado, CWD has since been documented in captive and/or free-ranging deer in 30 states and three Canadian provinces. To date, 376 captive or free-ranging cervids — including white-tailed deer, mule deer, red deer and elk — in 15 Texas counties have tested positive for CWD. For more information on previous detections visit the CWD page on the TPWD website.
4 MOST ENDANGERED WHITETAIL DESTINATIONS IN AMERICA
Mark Kenyon
MARK KENYON Jun 3, 2022
4 Most Endangered Whitetail Destinations in America
It would not be hyperbole to say that we’re quite possibly living in the “golden age” of whitetail deer hunting.
Deer populations might be higher now than ever before and, at least since records have been kept, bigger, older bucks have never been more numerous. Hunters smash world and state records every year. The good times sure seem to be rolling.
But this isn’t true across all aspects or locales within the whitetail range, nor is it guaranteed to remain true into the future. All good things can and do come to an end.
The future of deer and deer hunting, as is the case with almost every aspect of the natural world today, exists on a precipice. Serious threats like disease, habitat loss, resource management, and public opinion all loom on the horizon. Four specific threatened locations, in particular, stand out as representative of larger issues threatening our nation’s deer hunting future. These are, in our estimation, the four most endangered whitetail destinations in America.
Read on for an introduction to these special yet threatened whitetail locales. And find out what we can do as deer hunters and stewards of the land to address the challenges in these specific locations and across the nation.
SOUTHWESTERN WISCONSIN
Greatest Threat: Chronic Wasting Disease
Southwest Wisconsin, a world-renowned big buck destination, is also ground zero for chronic wasting disease. The 100% fatal neurological disease impacting whitetails and other deer species has now spread to 29 states across the country. CWD is widely recognized as possibly the greatest existential threat to the future of whitetail hunting, and no place is it more ubiquitous than Wisconsin’s driftless area.
In some regions here, hunters are observing population-level impacts and CWD prevalence rates have hit as high as 30%. In more practical terms, this means that some areas of the whitetail-crazy state of Wisconsin might be experiencing the beginnings of downward trends in populations due to CWD. Almost one in three deer tested in these areas are testing positive. While CWD’s large-scale impact on deer populations is no joke, an equally concerning risk is the impact that positive tests have on the desire to hunt and eat deer at all. While transmission from deer to humans hasn’t been documented, it’s theoretically possible—akin to what happened with Mad Cow Disease in the 1990s. For this reason, the CDC currently advises hunters not to consume venison from a CWD-positive deer. The result of all this is that a lot of hard-earned venison is getting thrown in the dump already, and things, hypothetically, stand to worsen.
This worst-in-class state of affairs is partly due to a passive approach to CWD management that Wisconsin adopted in 2012, moving away from their “earn a buck” rule, stopping targeted population controls, and making testing in known CWD areas only voluntary.
“Because of the passive management approach taken by Wisconsin, CWD is endemic to five southwest counties, has spread to surrounding counties, and has been found in 38 of the state’s 72 counties,” said southwestern Wisconsin resident, hunter, and land consultant Doug Duren. “In some areas in those counties where prevalence is being studied, over 50% of adult bucks and over 35% of adult does are CWD positive.”
In conversations with other area residents, Duren heard anecdotal reports of seeing fewer older deer and mature bucks, increasing numbers of late-stage infected animals in need of putting down, and already dead deer. “One member of a group of hunters with a lease in Iowa County, Wisconsin told me they decided to give up their lease and find opportunity elsewhere as every buck they killed in the past three years tested positive for CWD,” he said. “This is a cautionary tale.”
While there is no single simple fix to this problem, the recently introduced Chronic Wasting Disease Research and Management Act would designate $35 million in badly needed funding for CWD research and another $35 million for management and surveillance that would certainly help the situation in Wisconsin and beyond. Click here to let your senators know this is an issue of supreme importance to deer hunters in your state.
SNIP...
TEXAS
Greatest Threat: Captive Deer Industry
The great state of Texas sports one of the most robust and proud deer hunting cultures in the nation, but it’s also home to what some consider the greatest threat to deer hunting in all of America: the captive deer industry.
The debate around the captive deer industry is complicated, long-standing, and fraught, but the issues can be distilled into two main categories. First is the well-documented risk of spreading CWD by way of the transfer and sale of captive deer. Second is the negative impact that captive deer shooting facilities, and the media created around them, can have on the public perception of hunting in America and the North American Model of Conservation.
Texas is home to more captive deer facilities than any other state by a long shot, with 858 locations. The conditions present at such facilities, with high numbers of animals in close quarters, are well known to be a perfect storm for the spread of CWD. Not to mention the fact that given the transactional nature of captive deer breeding, many animals are sold and shipped across wide swaths of the country, potentially cross-contaminating new herds of deer all along the way.
While there have been increasing amounts of testing and monitoring of these herds for CWD, the effectiveness of these efforts are feared to be sub-par at best. “The profit motive is so great, it is common for deer breeders to hide infections, or simply not test, and thus spread the disease,” writes Whit Fosborg, CEO of the Theodore Roosevelt Conservation Partnership.
All of this makes Texas a likely ground zero for future CWD issues, a perfect example being a 2021 investigation that identified the release of more than 1,700 deer from seven Texas captive deer facilities that could have been exposed to chronic wasting disease. “Overwhelmingly, the CWD hot zone maps radiate from captive facilities across the state,” said Texas resident and bowhunter K.C. Smith.
Furthermore, the proliferation of captive deer facilities and “canned shooting preserves” threaten to shine a poor light on the larger hunting public, potentially hemorrhaging support for the free-range pursuit of whitetails in Texas and elsewhere. While the vast majority of non-hunters support hunting for food, those figures reverse when considering “trophy hunting.” Animals that are custom-bred to grow the largest antlers possible and then housed in high-fenced small enclosures and sold off to be shot by the highest bidder represents the most egregious example of what trophy hunting could be percieved as. Regardless of whether or not most high-fence facilities fit this description, the worst offenders are unfortunately what most people notice. We as whitetail hunters risk being defined by our most fringe element, especially in the Lone Star state.
It’s important to note that many of the high fence deer facilities in Texas very well might be well-managed and owned by good honest deer-loving people, Smith was quick to remind me. This is not as cut and dry of an issue as some non-Texans want to believe it is.
“I have an easy set of values to live by: don’t hurt people and don’t take their stuff,” he said. “However, when the deer ‘owned’ by another person are threatening to take away the future of my kids’ and everyone’s deer hunting, something must be done.”
Support for the Chronic Wasting Disease Research and Management Act will help just as much here as in Wisconsin, as would advocating for greater oversight of the captive deer industry in Texas and beyond.
***> TEXAS HISTORY OF CWD <***
Singeltary telling TAHC, that CWD was waltzing into Texas from WSMR around Trans Pecos region, starting around 2001, 2002, and every year, there after, until New Mexico finally shamed TAHC et al to test where i had been telling them to test for a decade. 2012 cwd was detected first right there where i had been trying to tell TAHC for 10 years.
***> Singeltary on Texas Chronic Wasting Disease CWD TSE Prion History <***
FRIDAY, JULY 15, 2022
Texas Chronic Wasting Disease CWD TSE Prion Positives Increase By 8 to 369 TOTAL Confirmed To Date
MONDAY, AUGUST 01, 2022
TEXAS Letter from Trophy Ranch to Governor Abbott about CWD TSE Prion
Proposed Amendments to CWD Zone Rules
Your opinions and comments have been submitted successfully.
Thank you for participating in the TPWD regulatory process.
THURSDAY, AUGUST 04, 2022
Texas Proposed Amendments to CWD Zone Rules Singeltary Submission
Governor Abbott on HOW TO LEGISLATE SPREADING CWD TO HELL AND BACK IN TEXAS $$$
i picked up on something that was said, there were several folks complaining that the breeders were getting picked on, and someone said something about trying to 'legislate' there way out of this. folks, this is terrible, i have seen this in other states, and it just spreads cwd even more. hell, it happened right here in Texas in the early days, that's why we are where were at now, you cannot let a bunch of Austin Legislative Socialites regulate CWD, just look what happened in Wisconsin. but i bet this attempted swaying of regulatory power shift from TPWD et al to the Texas Legislature in Austin is happening as we speak. we can't let this happen...
SUNDAY, JANUARY 22, 2017
Texas 85th Legislative Session 2017 Chronic Wasting Disease CWD TSE Prion Cervid Captive Breeder Industry
FRIDAY, JANUARY 27, 2017
TEXAS, Politicians, TAHC, TPWD, and the spread of CWD TSE Prion in Texas
SUNDAY, MAY 14, 2017
85th Legislative Session 2017 AND THE TEXAS TWO STEP Chronic Wasting Disease CWD TSE Prion, and paying to play $$$
Powerful Abbott appointee's lobbying sparks blowback in Legislature
In an ironic twist for Gov. Greg Abbott, who has made ethics reform an urgent political priority, the Texas House is taking aim at what critics call a "pay to play" culture among his appointees.
BY JAY ROOT MAY 12, 2017 12 AM
Houston billionaire Dan Friedkin is chairman of the Texas Parks and Wildlife Commission.
Texas Parks and Wildlife Commission
When Gov. Greg Abbott tapped one of his top campaign donors to become chairman of the Texas Parks and Wildlife Commission, he didn’t get a part-time appointee who would merely draft rules and implement conservation laws passed by the Legislature.
In Dan Friedkin, the governor got a Houston billionaire — with a team of privately funded lobbyists — willing to use his influence to ensure his wildlife interests are taken into account by the Legislature before they pass those laws, interviews and records show.
On the receiving end of that influence, and not in a happy way, is state Rep. Chris Paddie, R-Marshall. Paddie said a lobbyist working for Friedkin’s business empire, which includes a massive South Texas hunting ranch, has been working against his deer breeder management bill, which many large ranchers oppose. The state Parks and Wildlife Department oversees deer breeding regulations in Texas.
“Many times these appointees are well-heeled, very influential people,” Paddie said. “Overall, I feel that it’s inappropriate for an appointee of a board or commission to have personal lobbyists lobbying on issues related to that board or commission.”
Under Texas law, state agencies are barred from lobbying the Legislature. But the powerful people who oversee them aren’t.
If Paddie and dozens of his colleagues get their way, that practice soon will be a Class A misdemeanor.
Last weekend, Paddie attached a ban on appointee lobbying — which would apply to any issues intersecting with their state responsibilities — to an ethics bill that already had powerful friends of the governor in its crosshairs. The provision was adopted unanimously and the bill sailed out of the Texas House on a 91-48 vote Saturday.
The ethics bill, authored by Rep. Lyle Larson, R-San Antonio, would bar big campaign donors from getting appointed by governors in the first place. Anyone who contributed over $2,500 would be barred from serving on state boards and commissions.
Larson pointed to news articles documenting the amount of campaign money appointees have collectively given governors. Last year the San Antonio Express-News calculated that Abbott had received nearly $9 million from people he’s picked for appointed office; before that, a widely cited report from Texans for Public Justice found former Gov. Rick Perry had received $17 million from his own appointees.
Larson said 20 years from now, Texans will be reading the same stories about a future governor unless the Legislature does something about it now.
“We’ve read that article for the last three decades,” Larson said during a brief floor speech. “This is your opportunity to say, 'We need to stop this.' The most egregious ethics violation we’ve got in the state is the pay to play in the governor’s office.”
A prodigious fundraiser, Abbott has put plenty of big donors on prestigious boards and commissions. On the Parks and Wildlife Commission alone, he has installed three mega-donors — pipeline mogul Kelcy Warren, who’s given Abbott more than $800,000 over his statewide political career; Houston businessman S. Reed Morian, who has given $600,000; and Friedkin, who personally donated more than $700,000 — while his Gulf States Toyota PAC gave Abbott another $100,000, according to Ethics Commission records.
Passage of Larson’s HB 3305 represents an ironic twist for Abbott, who for the second session in a row has made ethics reform an urgent political priority — resulting in a bill that's now taking aim at his gubernatorial appointments. Abbott, who has made a habit of ignoring tough questions, hasn't made any public statements about the bill, and his office did not respond to multiple requests for comment.
Friedkin — whose wealth is estimated at $3.4 billion by Forbes — is the owner and CEO of Gulf States Toyota, founded in 1969, which has had the exclusive rights to distribute new Toyotas in Texas and four nearby states. He’d also been a mega-donor to former Gov. Rick Perry, who first appointed Friedkin to the Parks and Wildlife Commission in 2005. Abbott made Friedkin chairman of the commission in 2015.
Requests for comment from Friedkin's office went unanswered.
In addition to his public role as parks and wildlife chairman, a perch that gives him significant influence over deer management issues, Friedkin has private wildlife interests. He owns the sprawling Comanche Ranch in South Texas, according to published news accounts.
The January 2014 edition of Texas Wildlife, published by the Texas Wildlife Association, described Friedkin’s Comanche Ranch as “privately owned and privately hunted” and said it’s “in the business to produce as many trophy bucks as possible, without damaging the native habitat.”
The association, which advocates for private landowners and hunting rights, has locked horns with deer breeding interests at Parks and Wildlife and the Capitol. They compete against each other in the lucrative trophy deer hunting market — and the battle between them perennially spills into the rule-making process at the Parks and Wildlife Commission.
One of their battles centers on how captive deer are tagged so that game wardens and others can distinguish them from native deer. Current law requires a combination of tags and tattoos, and the ranchers and large landowners want to keep it that way. The breeders, meanwhile, favor tagging deer with microchips, which they contend are more accurate and foolproof.
The Wildlife Association said in a Facebook post that removing visible tag or tattoo requirements and allowing microchip tracking “creates real biosecurity risks and blurs ethical lines in the hunting community, as captive deer breeders are allowed to transport and release these animals to be co-mingled with pasture-born deer.” Proponents of the current system say tough rules on breeders are needed to keep out imported deer that may carry Chronic Wasting Disease, which has been found in Texas.
On the other side of the issue is the Texas Deer Association, which represents breeder interests. Executive Director Patrick Tarlton said opposition to his $1.6 billion industry stems less from environmental and health concerns and more from wealthy ranch owners who want to boost profits from trophy-seeking hunters. He notes that Chronic Wasting Disease has been found in both free range and captive deer.
Paddie sided with the breeders by filing House Bill 2855, which would allow breeders to track their deer with microchips instead of relying on physical tags that they say can be torn off.
No one identifying themselves as a Friedkin corporate lobbyist opposed the deer breeding bills during public hearings, according to House and Senate committee records published online.
Behind the scenes, it was a different story.
Paddie said his chief of staff reached out to Laird Doran, one of several lobbyists for Friedkin’s Gulf States Toyota, after hearing that he was trying to convince other legislators to help defeat Paddie's deer microchip bill.
“My chief called him and said, 'Hey, if you’ve got a problem with our bill why aren’t you talking to us?’ ” Paddie said. “He said he represented the Friedkin Group when that happened.”
According to an email from an aide to Sen. Craig Estes, R-Wichita Falls, who is carrying the deer breeding bill in the Senate, Doran also identified himself as a representative of the “Friedkin Group.” That’s the name of the consortium that contains Friedkin's Gulf States Toyota, according to the company’s Linked-In page. He told Estes’ aide that the Friedkin group was opposed to any bill that would “remove requirements for (deer) ear tags,” the senator’s office confirmed.
It’s not clear exactly which Friedkin interests Doran was advancing. Doran is registered at the Texas Ethics Commission with a single entity — Gulf States Toyota — and the agency has no record of a lobbyist working for an entity or individual with the name Friedkin in it, the commission confirmed Wednesday afternoon.
However, Doran checked a variety of non-automotive subject areas in which he is lobbying during this legislative session on behalf of Friedkin’s lucrative distributorship, including “animals,” “parks & wildlife,” “state agencies, boards & commissions,” “environment” and more, his detailed lobby disclosures show.
Doran, director of government relations and senior counsel at the Friedkin Group, did not return phone and email messages left by The Texas Tribune.
Estes said he didn’t have a problem with a governor's appointee engaging in lobbying on issues that affected their private interests, as long as they keep that separate from their state roles.
“I don’t think they should be barred from expressing their views as long as they’re careful to say these are my views, not the views of the agency I’m representing,” Estes said.
But Tarlton, the deer association director, said Friedkin’s use of lobbyists to oppose deer breeders in the Legislature gives the breeders' opponents a huge advantage.
“I think that if the commissioner of Texas Parks and Wildlife is actively lobbying against an industry which his department directly oversees, it absolutely sets up an unfair and closed system of government,” Tarlton said. “The commission is supposed to be the unbiased and equitable oversight for everything wildlife.”
Paddie hopes his amendment to Larsen's ethics bill will even the playing field. He referred to the wealthy Parks and Wildlife chairman (see the 2:29:00 mark in this recorded exchange) when he tacked the appointee-lobbying provision onto Larson’s bill.
Paddie said he’s not singling out anyone. He said it would apply to other powerful gubernatorial appointees in a position to do the same.
“I could have named any number of examples as far as the agencies in particular,” Paddie said. “I want to stop it if anyone serving on any agency is doing this.”
Ryan Murphy contributed to this report.
Disclosure: The Texas Wildlife Association, Texas Parks and Wildlife Department and Gulf States Toyota have been financial supporters of The Texas Tribune. A complete list of Tribune donors and sponsors is available here.
TUESDAY, AUGUST 02, 2016
TEXAS TPWD Sets Public Hearings on Deer Movement Rule Proposals in Areas with CWD Rule Terry S. Singeltary Sr. comment submission
SUNDAY, MAY 22, 2016
TEXAS CWD DEER BREEDERS PLEA TO GOVERNOR ABBOTT TO CIRCUMVENT TPWD SOUND SCIENCE TO LET DISEASE SPREAD
Wednesday, May 04, 2016
TPWD proposes the repeal of §§65.90 -65.94 and new §§65.90 -65.99 Concerning Chronic Wasting Disease - Movement of Deer Singeltary Comment Submission
Terry S. Singeltary Sr. Your opinions and comments have been submitted successfully. Thank you for participating in the TPWD regulatory process.
Wednesday, October 28, 2015
Interim Chronic Wasting Disease Response Rules Comment online through 07:00 a.m. November 5, 2015
SUNDAY, AUGUST 23, 2015
Subject: Chronic Wasting Disease CWD TSE Prion and how to put lipstick on a pig and take her to the dance in Texas I was listening to a radio show the other day here in the Galveston bay area, and outdoor show, they had a breeder or someone from the industry on, and I was amazed at the false information he was spewing. the part about the poor little girl with her pet deer crying in the breeder pen, ......
cry me a friggen river, they are raising the damn deer to put in a pen to slaughter, or to breed for that purpose, AND you ought to see a human die from this shit. my mother did everything Linda Blair did in that movie the exorcist except spin her head 360 degrees. she DID levitate in bed because she would jerk so bad, where it took three grown strong adults to hold her down to keep her from hurting herself, all the while screaming God why can’t I stop this. so cry me a fucking river on a damn deer they are raising to have slaughtered, but whine because the TPWD et al are going to kill it to try and prevent the spread of disease cwd. if the TPWD et al had a better way of confirming or not whether those cervid had CWD, they would do it. the live tests they have to date do not work 100%, so there for they have not been validated. oh that’s fine with the pen owners, but it’s not fine for Texas. you don’t want a cwd test that just works part of the time. it’s total ignorance out there now, and they will put lipstick on this pig and take her to the dance, just like TAHC did with mad cow disease, and that’s well documented. they will change what ever law to meet their needs$$$ I will agree with this much of what the industry said this morning, that cwd has been in Texas for a long time, and in the pens to, and that the TAHC has not tested enough, that much he got correct. I have been saying this year, after year, after year, since back to 2001, to the TAHC, and told them exactly where they should be testing back in 2001, and then year after year after year, up and until 2012, where they finally did test there in enough numbers to find it a decade later, exactly where I been saying it was. the cwd deer have been waltzing across Texas from there for over a decade. it does not matter if I am pro-pen or not. that will not and does not change the science. why in the hell did they speak about the 4 confirmed deer from that index herd, yes, I said 4 now. why is not the TAHC TPWD telling that to the public now. why did not that guy today speak of 4? all the newspapers are reporting it, and I ask about the 4th case weeks and weeks ago? where is that information at on TAHC site? I am a meat eater, I am pro-hunt, and extremely pro-gun, I am however anti-stupid and anti-prion, prions can kill you, I don’t want to eat prions, you should not either. but here is the kicker, you eat meat infected with CWD TSE prion, your exposed, however you never go clinical in your life........BBBUT, your exposed and if you go on to have surgical, dental, tissue, blood donations, etc. you risk exposing my family and others...I will simply post this one short abstract of an old study the late great Dr. Gibbs...
Texas 84th Legislative Session Sunday, December 14, 2014
*** TEXAS 84th Legislature commencing this January, deer breeders are expected to advocate for bills that will seek to further deregulate their industry
TUESDAY, DECEMBER 16, 2014
Texas 84th Legislature 2015 H.R. No. 2597 Kuempel Deer Breeding Industry TAHC TPWD CWD TSE PRION
expand this to see all breeder cwd, and then think of what they have released at release sites...
“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD.
TAHC Chapter 40, Chronic Wasting Disease Terry Singeltary Comment Submission
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
Title: Exploring the possibility of CWD transmission through artificial insemination of semen from CWD positive bucksAuthor
Submitted to: North American Deer Farmer Publication Type: Trade Journal Publication Acceptance Date: 7/1/2022 Publication Date: 7/20/2022 Citation: Cassmann, E.D., Greenlee, J.J. 2022. Exploring the possibility of CWD transmission through artificial insemination of semen from CWD positive bucks. North American Deer Farmer. p. 107-109. Interpretive Summary: Technical Abstract: Artificial insemination is a popular method for herd management and reproduction in the cervid farming industry. There are numerous benefits including increased access to superior genetics, convenience, and increased farm revenue. In this article, we summarize the research that is underway to determine if semen from a CWD infected buck can transmit the disease. Some research has already been performed on the reproductive transmission of CWD in cervids. Scientists from Colorado State University used Muntjac deer to demonstrate that CWD positive does could transmit CWD to their fawns (1). In the study, Muntjac does were bred to CWD negative bucks. At the time of breeding, does were either in the early or late stage of CWD infection. Fawns from both doe groups were positive for CWD. More recent laboratory experiments on semen from CWD positive bucks have demonstrated the presence of CWD prions (2). Researchers used an amplification technique called PMCA (protein misfolding cyclic amplification). The technique amplifies low levels of CWD prions to a point where conventional antibody methods can detect them. The caveat to the discovery of CWD prions in semen is that we’re unsure if the amount of CWD prions in semen is biologically relevant. In other words, is the level of CWD prions in semen sufficient to be infectious and cause disease in deer? In our present study, we are trying to answer that question. We obtained semen from a CWD positive buck. The semen was confirmed to be PMCA positive. For the study, we used the transcervical insemination method in three does. As of June 19th, a single fawn was born. Shortly after birth the fawn was separated to prevent environment CWD exposure. We are assessing both the does and the fawn for the development of CWD. The experiment is expected to last several years, and periodic sampling will help monitor progress. A second phase of the study will investigate the absolute susceptibility of white-tailed deer does to CWD after transcervical and/or vaginal exposure to large amounts of CWD prions. We plan to expose two does to brain suspension from a CWD positive deer. These does will also be monitored long term for the development of disease. If these does remain negative, it would indicate that CWD transmission to the dam is highly unlikely via reproductive tract exposure. TUESDAY, DECEMBER 31, 2019 In Vitro detection of Chronic Wasting Disease (CWD) prions in semen and reproductive tissues of white tailed deer bucks (Odocoileus virginianus TEXAS CWD, Have you been ThunderStruck, deer semen, straw bred bucks, super ovulation, and the potential TSE Prion connection, what if? SUNDAY, FEBRUARY 16, 2020 ***> Jerking for Dollars, Are Texas Politicians and Legislators Masturbating Deer For Money, and likely spreading CWD TSE Prion? THURSDAY, JULY 10, 2014 supercalifragilisticexpialidocious or superovulationcwdtsepriondocious ? (It was noted with concern that hormone extracts could be manufactured by a veterinary surgeon for administration to animals under his care without any Medicines Act Control.) PITUITARY EXTRACT This was used to help cows super ovulate. *** This tissue was considered to be of greatest risk of containing BSE and consequently transmitting the disease. *** Proposed Amendments to CWD Zone Rules Your opinions and comments have been submitted successfully. Thank you for participating in the TPWD regulatory process. THURSDAY, AUGUST 04, 2022 Texas Proposed Amendments to CWD Zone Rules Singeltary Submission FRIDAY, APRIL 30, 2021 Should Property Evaluations Contain Scrapie, CWD, TSE PRION Environmental Contamination of the land? ***> Confidential!!!! ***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!! ---end personal email---end...tss WEDNESDAY, MAY 17, 2017 *** Chronic Wasting Disease CWD TSE Prion aka Mad Deer Disease and the Real Estate Market Land Values *** MONDAY, MARCH 05, 2018 TRUCKING AROUND AND SPREADING CHRONIC WASTING DISEASE CWD TSE PRION VIA MOVEMENT OF CERVID AND TRANSPORTATION VEHICLES TUESDAY, JUNE 28, 2022 TAHC PROPOSES CHANGES TO VOLUNTARY CWD PROGRAM CHAPTER 40, CHRONIC WASTING DISEASE SINGELTARY SUBMISSION JUNE 28, 2022 ***> TEXAS HISTORY OF CWD <*** Singeltary telling TAHC, that CWD was waltzing into Texas from WSMR around Trans Pecos region, starting around 2001, 2002, and every year, there after, until New Mexico finally shamed TAHC et al to test where i had been telling them to test for a decade. 2012 cwd was detected first right there where i had been trying to tell TAHC for 10 years. ***> Singeltary on Texas Chronic Wasting Disease CWD TSE Prion History <*** |
A third issue is the accuracy of mortality reporting. Department records indicate that for each of the last five years an average of 26 deer breeders have reported a shared total of 159 escapes. Department records for the same time period indicate an average of 31 breeding facilities reported a shared total of 825 missing deer (deer that department records indicate should be present in the facility, but cannot be located or verified).
WEDNESDAY, DECEMBER 14, 2022
CHRONIC WASTING DISEASE CWD TSE PRION UPDATE DECEMBER 14, 2022
Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
Comment from Singeltary, Terry Posted by the Animal and Plant Health Inspection Service on Mar 11, 2021
Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
Greetings APHIS et al, i would kindly like to comment on Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004.
Greetings APHIS et al, i would kindly like to comment on Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004.
***> 1st and foremost your biggest problem is 'VOLUNTARY'! AS with the BSE 589.2001 FEED REGULATIONS, especially since it is still voluntary with cervid, knowing full well that cwd and scrapie will transmit to pigs by oral route. VOLUNTARY DOES NOT WORK! all animal products should be banned and be made mandatory, and the herd certification program should be mandatory, or you don't move cervid. IF THE CWD HERD CERTIFICATION IS NOT MANDATORY, it will be another colossal tse prion failure from the start.
***> 2nd USA should declare a Declaration of Extraordinary Emergency due to CWD, and all exports of cervid and cervid products must be stopped internationally, and there should be a ban of interstate movement of cervid, until a live cwd test is available.
***> 3rd Captive Farmed cervid ESCAPEES should be made mandatory to report immediately, and strict regulations for those suspect cwd deer that just happen to disappear. IF a cervid escapes and is not found, that farm should be indefinitely shut down, all movement, until aid MIA cervid is found, and if not ever found, that farm shut down permanently.
***> 4th Captive Farmed Cervid, INDEMNITY, NO MORE Federal indemnity program, or what i call, ENTITLEMENT PROGRAM for game farm industry. NO MORE BAIL OUTS FROM TAX PAYERS. if the captive industry can't buy insurance to protect not only themselves, but also their customers, and especially the STATE, from Chronic Wasting Disease CWD TSE Prion or what some call mad deer disease and harm therefrom, IF they can't afford to buy that insurance that will cover all of it, then they DO NOT GET A PERMIT to have a game farm for anything. This CWD TSE Prion can/could/has caused property values to fall from some reports in some places. roll the dice, how much is a state willing to lose?
***> 5th QUARANTINE OF ALL FARMED CAPTIVE, BREEDERS, URINE, ANTLER, VELVET, SPERM, OR ANY FACILITY, AND THEIR PRODUCTS, that has been confirmed to have Chronic Wasting Disease CWD TSE Prion, the QUARANTINE should be for 21 years due to science showing what scrapie can do. 5 years is NOT near long enough. see; Infectious agent of sheep scrapie may persist in the environment for at least 16 to 21 years.
***> 6th America BSE 589.2001 FEED REGULATIONS CWD TSE Prion
***> 7TH TRUCKING TRANSPORTING CERVID CHRONIC WASTING DISEASE TSE PRION VIOLATING THE LACEY ACT
***> 8TH ALL CAPTIVE FARMING CERVID OPERATIONS MUST BE INSURED TO PAY FOR ANY CLEAN UP OF CWD AND QUARANTINE THERE FROM FOR THE STATE, NO MORE ENTITLEMENT PROGRAM FOR CERVID GAME FARMING PAY TO PLAY FOR CWD TSE PRION OFF THE TAX PAYERS BACK.
***> 9TH ANY STATE WITH DOCUMENTED CWD, INTERSTATE, NATIONAL, AND INTERNATIONAL MOVEMENT OF ALL CERVID, AND ALL CERVID PRODUCTS MUST BE HALTED!
***> 10TH BAN THE SALE OF STRAW BRED BUCKS AND ALL CERVID SEMEN AND URINE PRODUCTS
***> 11th ALL CAPTIVE FARMED CERVID AND THEIR PRODUCTS MUST BE CWD TSE PRION TESTED ANNUALLY AND BEFORE SALE FOR CWD TSE PRION
SEE FULL SCIENCE REFERENCES AND REASONINGS ;
***> 1st and foremost your biggest problem is 'VOLUNTARY'!
''APHIS created a cooperative, voluntary Federal-State-private sector CWD Herd Certification Program designed to identify farmed or captive herds infected with CWD.''
key word failure is 'voluntary'.
WE know for a fact now that voluntary does NOT WORK!
AS with the BSE 589.2001 FEED REGULATIONS (see , another colossal failure, and proven to be a sham, especially since it is still voluntary with cervid, knowing full well that cwd and scrapie will transmit to pigs by oral route. VOLUNTARY DOES NOT WORK! all animal products should be banned and be made mandatory, and the herd certification program should be mandatory, or you don't move cervid. IF THE CWD HERD CERTIFICATION IS NOT MANDATORY, it will be another colossal tse prion failure from the start.
***> 2nd USA should declare a Declaration of Extraordinary Emergency due to CWD, and all exports of cervid and cervid products must be stopped internationally, and there should be a ban of interstate movement of cervid, until a live cwd test is available.
***> 3rd Captive Farmed cervid ESCAPEES should be made mandatory to report immediately, and strict regulations for those suspect cwd deer that just happen to disappear. IF a cervid escapes and is not found, that farm should be indefinitely shut down, all movement, until aid MIA cervid is found, and if not ever found, that farm shut down permanently. ...snip...see full text submission with science references...TSS
Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification
APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment SubmissionComment from Singeltary Sr., Terry
Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022MAD COW FEED BAN AND CERVID FEED, WHAT IF?e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;Product manufactured from 02/01/2005 until 06/06/2006RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as"Do not feed to ruminants".VOLUME OF PRODUCT IN COMMERCE 125 tonsDISTRIBUTION AL and FLEND OF ENFORCEMENT REPORT FOR AUGUST 2, 2006Friday, December 14, 2012DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012snip...In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.Animals considered at high risk for CWD include:1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.snip...OIE atypical BSEGiven that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.OIE Conclusions on transmissibility of atypical BSE among cattleGiven that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.OIE Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 201934 Scientific Commission/September 20193. Atypical BSEThe Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.4. Definitions of meat-and-bone meal (MBM) and greavessnip...REFERENCESSNIP...END SEE FULL TEXT;Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), is the third potentially zoonotic PD (with BSE and L-type BSE), thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.2.3.2. New evidence on the zoonotic potential of atypical BSE and atypical scrapie prion strainsOlivier Andreoletti, INRA Research Director, Institut National de la Recherche Agronomique (INRA) – École Nationale Vétérinaire de Toulouse (ENVT), invited speaker, presented the results of two recently published scientific articles of interest, of which he is co-author:‘Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice’ (MarinMoreno et al., 2020) and ‘The emergence of classical BSE from atypical/Nor98 scrapie’ (Huor et al., 2019).In the first experimental study, H-type and L-type BSE were inoculated into transgenic mice expressing all three genotypes of the human PRNP at codon 129 and into adapted into ARQ and VRQ transgenic sheep mice. The results showed the alterations of the capacities to cross the human barrier species (mouse model) and emergence of sporadic CJD agents in Hu PrP expressing mice: type 2 sCJD in homozygous TgVal129 VRQ-passaged L-BSE, and type 1 sCJD in homozygous TgVal 129 and TgMet129 VRQ-passaged H-BSE.This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author AffiliationsIn mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.Prions in Skeletal Muscles of Deer with Chronic Wasting DiseaseHere bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting diseaseNathaniel D. Denkers ,Clare E. Hoover ,Kristen A. Davenport,Davin M. Henderson,Erin E. McNulty,Amy V. Nalls,Candace K. Mathiason,Edward A. HooverPublished: August 20, 2020We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.cwd scrapie pigs oral routes***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <******> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.CONFIDENTIALEXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHYLINE TO TAKE3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:-"There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.DO Hagger RM 1533 MT Ext 3201While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....Monday, November 14, 2022Prion Diseases in Dromedary Camels (CPD) 2022 Review***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.WE know now, and we knew decades ago, that 5.5 grams of suspect feed in TEXAS was enough to kill 100 cows.look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;Risk of oral infection with bovine spongiform encephalopathy agent in primatesCorinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe DeslysSummary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.snip...BSE bovine brain inoculum100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mgPrimate (oral route)* 1/2 (50%)Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%)RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)PrPres biochemical detectionThe comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal.Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inoculaPublished online January 27, 2005It is clear that the designing scientists mustalso have shared Mr Bradley’s surprise at the results because all the doselevels right down to 1 gram triggered infection.6. It also appears to me that Mr Bradley’s answer (that it would take less than say 100grams) was probably given with the benefit of hindsight; particularly if oneconsiders that later in the same answer Mr Bradley expresses his surprise that itcould take as little of 1 gram of brain to cause BSE by the oral route within thesame species. This information did not become available until the "attack rate"experiment had been completed in 1995/96. This was a titration experimentdesigned to ascertain the infective dose. A range of dosages was used to ensurethat the actual result was within both a lower and an upper limit within the studyand the designing scientists would not have expected all the dose levels to triggerinfection. The dose ranges chosen by the most informed scientists at that timeranged from 1 gram to three times one hundred grams. It is clear that the designingscientists must have also shared Mr Bradley’s surprise at the results because all thedose levels right down to 1 gram triggered infection.a review of banned mad cow feed in USA;BANNED MAD COW FEED IN COMMERCE IN ALABAMADate: September 6, 2006 at 7:58 am PST PRODUCTa) EVSRC Custom dairy feed, Recall # V-130-6;b) Performance Chick Starter, Recall # V-131-6;c) Performance Quail Grower, Recall # V-132-6;d) Performance Pheasant Finisher, Recall # V-133-6.CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.REASONDairy and poultry feeds were possibly contaminated with ruminant based protein.VOLUME OF PRODUCT IN COMMERCE 477.72 tonsDISTRIBUTION AL______________________________http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.htmlPRODUCT Bulk custom dairy pre-mixes,Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.VOLUME OF PRODUCT IN COMMERCE 350 tonsDISTRIBUTION AL and MS______________________________PRODUCTa) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb. bags, Recall # V-121-6;b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6;c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6;d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6;e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6;f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6;g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bagsDISTRIBUTION AL, GA, MS, and TNEND OF ENFORCEMENT REPORT FOR AUGUST 9, 2006###http://www.fda.gov/bbs/topics/ENFORCE/2006/ENF00964.htmlSubject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006Date: August 6, 2006 at 6:16 pm PST PRODUCTa) CO-OP 32% Sinking Catfish, Recall # V-100-6;b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6;c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6;h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6;i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;j) CO-OP LAYING CRUMBLES, Recall # V-109-6;k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6;l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODEProduct manufactured from 02/01/2005 until 06/06/2006RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".VOLUME OF PRODUCT IN COMMERCE 125 tonsDISTRIBUTION AL and FLEND OF ENFORCEMENT REPORT FOR AUGUST 2, 2006###http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.htmlMAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II______________________________PRODUCTa) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;d) Feather Meal, Recall # V-082-6 CODEa) Bulkb) Nonec) Bulkd) BulkRECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.REASONPossible contamination of animal feeds with ruminent derived meat and bone meal.VOLUME OF PRODUCT IN COMMERCE 10,878.06 tonsDISTRIBUTION NationwideEND OF ENFORCEMENT REPORT FOR July 12, 2006###http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007Date: March 21, 2007 at 2:27 pm PSTRECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II___________________________________PRODUCTBulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007CODECattle feed delivered between 01/12/2007 and 01/26/2007RECALLING FIRM/MANUFACTURERPfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.Firm initiated recall is ongoing.REASONBlood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.VOLUME OF PRODUCT IN COMMERCE42,090 lbs.DISTRIBUTIONWI___________________________________PRODUCTCustom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007CODEThe firm does not utilize a code - only shipping documentation with commodity and weights identified.RECALLING FIRM/MANUFACTURERRangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.REASONProducts manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.VOLUME OF PRODUCT IN COMMERCE9,997,976 lbs.DISTRIBUTIONID and NVEND OF ENFORCEMENT REPORT FOR MARCH 21, 2007http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***Sunday, March 20, 2016Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary SubmissionSEE MAD COW FEED VIOLATIONS AFER MAD COW FEED VIOLATIONS ;Tuesday, April 19, 2016Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards Singeltary Comment SubmissionMONDAY, OCTOBER 10, 2022Docket No: 2002N-0273 (formerly Docket No. 02N-0273) Substances Prohibited From Use in Animal Food and Feed Scientists Comments December 20, 2005This information is critical, and should continue to be collected.The TSE prion is spreading across the USA in Cervid as in CWD TSE Prion.The mad cow surveillance, feed ban, testing, and SRM removal there from, has been, and still is, a terrible failure.WE know that the USA Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) of August 1997 was/is a colossal failure, and proven to be so year after year, decade after decade, and this was just admitted by the FDA et al (see below FDA Reports on VFD Compliance Sept. 2019 report).God, all these decades you hear from all the warning letters on SRM that were released to the public for consumption, that even if they did eat a SRM, the BSE Feed Regulation (21 CFR 589.2000) of August 1997 would save that tissue from that animal from having a TSE Prion, was nothing but lies. what about those children all across the USA that were fed the most high risk cattle for mad cow disease, i.e. dead stock downer cows via the USDA School lunch program, who will watch those kids for the next 50 years for cjd tse prion aka mad cow disease, let alone all the folks consuming SRMs that have been exposed to mad cow type disease in different livestock species, due to the fact the USA colossal failure of the BSE Feed Regulation (21 CFR 589.2000) of August 1997. it's all documented below, see for yourself; SUNDAY, SEPTEMBER 1, 2019 FDA Reports on VFD ComplianceTuesday, September 10, 2019FSIS [Docket No. FSIS–2019–0021] Notice of Request To Renew an Approved Information Collection: Specified Risk Materials Singeltary Submission
MAD COW FEED, AND CWD, WHAT IF???
FRIDAY, FEBRUARY 17, 2023
Alabama Third Case of CWD Confirmed in Lauderdale County
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
CHRONIC WASTING DISEASE ZOONOSIS ZOONOTIC POTENTIAL, OR HAS IT ALREADY HAPPENED, WHAT IF?
WHAT WILL CWD PATHOLOGY IN HUMANS LOOK LIKE?
WILL IT LOOK LIKE nvCJD, OF THE EARLY BSE DAYS?
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From: Sent: Sunday, September 29, 2002 10:15 AM
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
> However, to date, no CWD infections have been reported in people.
sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.
if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
sporadic = 54,983 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic
spontaneous = 325,650 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
> However, to date, no CWD infections have been reported in people.
key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ References:
Dear Terry,
An excellent piece of review as this literature is desperately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler ===============
''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
Table 9 presents the results of an analysis of these data.
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
snip...
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf
http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf
Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk
BSE Inquiry Steve Dealler
Management In Confidence
BSE: Private Submission of Bovine Brain Dealler
snip...see full text;
MONDAY, FEBRUARY 25, 2019
***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019
***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''
***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***
***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<***
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<***
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases).
Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.
==============
PRION 2015 CONFERENCE
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
2004
Jeff Swann and his Mom, cwd link... sporadic CJD?, CBC NEWS Jeff Schwan sCJD, CWD, and Professor Aguzzi on BSE and sporadic CJD
????: CBCnews
Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.
Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla
aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany
Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.
Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPCfollowed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.
Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPressignature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPresdeposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPresin immunoblot, prion seeding activity, and PrPresdeposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.
Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.
Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada.
Grant number: 1R01NS121016-01; 201,600,023
Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.
Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD
Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha
aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada
Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.
Material and Methods: Transgenic mice overexpressing human PrPChomozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.
Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPCwith deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.
Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.
Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.
Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.
Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.
PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS
Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease.
Samia Hannaouia, Ginny Chenga, Wiebke Wemheuerb, Walter J. Schulz-Schaefferb, Sabine Gilcha, and Hermann M. Schätzla aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine & Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bInstitute of Neuropathology, Medical Faculty, Saarland University, Homburg/Saar, Germany
Aims: Chronic wasting disease (CWD) is a prion disease of cervids. Its rapid geographic expansion, shedding of infectivity and persistence in the environment for many years are of concern for humans. Here, we provide the first evidence by transmission experiments to different transgenic mouse models and bank voles that Cynomolgus macaques inoculated via different routes with CWD-positive cervid tissues harbor infectious prions that elicit clinical disease in rodents.
Material and Methods: We used tissue materials from macaques inoculated with CWD to inoculate transgenic mice overexpressing cervid PrPC followed by transmission into bank voles. We used RT-QuIC, immunoblot and PET blot analysis to assess brains, spinal cords, and tissues of the gastrointestinal tract (GIT) for the presence of prions.
Results: Our results show that of the macaque materials that induced clinical disease in transgenic mice,73% were from the CNS (46% spinal cord and 27% brain), and 27% were from the spleen, although attack rates were low around 20%. Clinical mice did not display PK-resistant PrPSc(PrPres) in immunoblot, but showed low-levels of prion seeding activity. Transmission into bank voles from clinical transgenic mice led to a 100% attack rate with typical PrPres signature in immunoblot, which was different from that of voles inoculated directly with CWD or scrapie prions. High-level prion seeding activity in brain and spinal cord and PrPres deposition in the brain were present. Remarkably, we also found prion seeding activity in GIT tissues of inoculated voles. Second passage in bank voles led to a 100% attack rate in voles inoculated with brain, spinal cord and small intestine material from first round animals, with PrPres in immunoblot, prion seeding activity, and PrPres deposition in the brain. Shortened survival times indicate adaptation in the new host. This also shows that prions detected in GIT tissues are infectious and transmissible. Transmission of brain material from sick voles back to cervidized mice revealed transmission in these mice with a 100% attack rate, and interestingly, with different biochemical signature and distribution in the brain.
Conclusions: Our findings demonstrate that macaques, considered the best model for the zoonotic potential of prions, were infected upon CWD challenge, including oral one. The disease manifested as atypical in macaques and transgenic mice, but with infectivity present at all times, as unveiled in the bank vole model with an unusual tissue tropism.
Funded by: The National Institutes of Health, USA, and the Alberta Prion Research Institute/Alberta Innovates Canada. Grant number: 1R01NS121016-01; 201,600,023
Acknowledgement: We thank Umberto Agrimi, Istituto Superiore di Sanità, Rome, Italy, and Michael Beekes, Robert-Koch Institute Berlin, Germany, for providing the bank vole model. We thank the University of Calgary animal facility staff and Dr. Stephanie Anderson for animal care.
Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potential of CWD
Samia Hannaouia, Irina Zemlyankinaa, Sheng Chun Changa, Maria Immaculata Arifina, Vincent Béringueb, Debbie McKenziec, Hermann M. Schatzla, and Sabine Gilcha
aDepartment of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine; Hotchkiss Brain Institute; University of Calgary, Calgary, Canada; bUniversité Paris-Saclay, INRAE, UVSQ, VIM, Jouy-en-Josas, France; cDepartment of Biological Sciences, Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Canada
Aims: Chronic wasting disease (CWD), a prion disease of cervids, spreads efficiently among wild and farmed animals. Potential transmission to humans of CWD is a growing concern due to its increasing prevalence. Here, we aimed to determine the zoonotic potential of CWD using a mouse model for human prion diseases.
Material and Methods: Transgenic mice overexpressing human PrPC homozygous for methionine at codon 129 (tg650) were inoculated intracerebrally with brain homogenates of white-tailed deer infected with Wisc-1/CWD1 or 116AG CWD strains. Mice were monitored for clinical signs and were euthanized at terminal disease. Brains were tested by RT-QuIC, western blot upon PK digestion, and immunohistochemistry; fecal homogenates were analyzed by RT-QuIC. Brain/spinal cord and fecal homogenates of CWD-inoculated tg650 mice were inoculated into tg650 mice or bank voles. Brain homogenates of bank voles inoculated with fecal homogenates of CWD-infected tg650 mice were used for second passage in bank voles.
Results: Here, we provide the strongest evidence supporting the zoonotic potential of CWD prions, and their possible phenotype in humans. Inoculation of mice expressing human PrPC with deer CWD isolates (strains Wisc-1 and 116AG) resulted in atypical clinical manifestations in > 75% of the mice, with myoclonus as leading clinical sign. Most of tg650 brain homogenates were positive for seeding activity in RT-QuIC. Clinical disease and presentation was transmissible to tg650 mice and bank voles. Intriguingly, protease-resistant PrP in the brain of tg650 mice resembled that found in a familial human prion disease and was transmissible upon passage. Abnormal PrP aggregates upon infection with Wisc-1 were detectable in thalamus, hypothalamus, and midbrain/pons regions.
Unprecedented in human prion disease, feces of CWD-inoculated tg650 mice harbored prion seeding activity and infectious prions, as shown by inoculation of bank voles and tg650 with fecal homogenates.
Conclusions: This is the first evidence that CWD can infect humans and cause disease with a distinctive clinical presentation, signature, and tropism, which might be transmissible between humans while current diagnostic assays might fail to detect it. These findings have major implications for public health and CWD-management.
Funded by: We are grateful for financial support from the Natural Sciences and Engineering Research Council of Canada, the National Institutes of Health, Genome Canada, and the Alberta Prion Research Institute. SG is supported by the Canada Research Chairs program.
Acknowledgement: We thank Dr. Trent Bollinger, WCVM, University of Saskatchewan, Saskatoon, Canada, for providing brain tissue from the WTD-116AG isolate, Dr. Stéphane Haïk, ICM, Paris, France, for providing brain tissue from vCJD and sCJD cases, and Dr. Umberto Agrimi, Istituto Superiore di Sanità, Italy, for the bank vole model. We thank animal facility staff for animal care, Dr. Stephanie Anderson for veterinary oversight, and Yo-Ching Cheng for preparing recombinant PrP substrates. Thank you to Dr. Stephanie Booth and Jennifer Myskiw, Public Health Agency of Canada, Canada.
The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons
Eric Cassmanna, Xu Qib, Qingzhong Kongb, and Justin Greenleea
aNational Animal Disease Center, Agricultural Research Service, US Department of Agriculture, Ames, IA, USA bDepartments of Pathology, Neurology, National Center for Regenerative Medicine, and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA
Aims: Evaluate the zoonotic potential of the raccoon passaged chronic wasting disease (CWD) agent in humanized transgenic mice in comparison with the North American CWD agent from the original white-tailed deer host.
Material and Methods: Pooled brain material (GG96) from a CWD positive herd was used to oronasally inoculate two white-tailed deer with wild-type prion protein genotype and intracranially inoculate a raccoon. Brain homogenates (10% w/v) from the raccoon and the two white-tailed deer were used to intracranially inoculate separate groups of transgenic mice that express human prion protein with methionine (M) at codon 129 (Tg40h). Brains and spleens were collected from mice at experimental endpoints of clinical disease or approximately 700 days post-inoculation. Tissues were divided and homogenized or fixed in 10% buffered neutral formalin. Immunohistochemistry, enzyme immunoassay, and western blot were used to detect misfolded prion protein (PrPSc) in tissue.
Results: Humanized transgenic mice inoculated with the raccoon passaged CWD agent from white-tailed deer exhibited a 100% (12/12) attack rate with an average incubation period of 605 days. PrPSc was detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPSc also was detected in brain tissue by western blot and immunohistochemistry. No PrPSc was detected in the spleens of mice inoculated with the raccoon passaged CWD agent. Humanized mice inoculated with the CWD agent from white-tailed deer did not have detectable PrPSc using conventional immunoassay techniques.
Conclusions: The host range of the CWD agent from white-tailed deer was expanded in our experimental model after one passage through raccoons.
Funded by: This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or preparation of the manuscript.
Acknowledgement: We thank Quazetta Brown, Lexi Frese, Rylie Frese, Kevin Hassall, Leisa Mandell, and Trudy Tatum for providing excellent technical support to this project.
Stable and highly zoonotic cervid prion strain is possible
Manuel Camacho, Xu Qi, Liuting Qing, Sydney Smith, Jieji Hu, Wanyun Tao, Ignazio Cali, and Qingzhong Kong Department of Pathology, Case Western Reserve University, Cleveland, USA
Aims: Whether CWD prions can infect humans remains unclear despite the very substantial scale and long history of human exposure of CWD in some areas. Multiple in vitro conversion experiments and in vivo animal studies suggest that the CWD-to-human transmission barrier is not unbreakable. A major public health concern on CWD zoonosis is the emergence of highly zoonotic CWD strains. We aim to address the question of whether highly zoonotic CWD strains are possible.
Material and Methods: We inoculated a few sCJD brain samples into cervidized transgenic mice, which were intended as negative controls for bioassays of brain tissues from sCJD cases who had hunted or consumed vension from CWD-endemic states. Some of these mice became infected and their brain tissues were further examined by serial passages in humanized or cervidized mice.
Results: Passage of sCJDMM1 in transgenic mice expressing elk PrP (Tg12) resulted in a ‘cervidized’ CJD strain that we termed CJDElkPrP. We observed 100% transmission of CJDElkPrP in transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrP two more times in the Tg12 mice. We found that such second and third passage CJDElkPrP prions also led to 100% infection in the Tg40h mice. In contrast, we and others found zero or poor transmission of natural elk CWD isolates in humanized mice, despite that natural elk CWD isolates and CJDElkPrP share the same elk PrP sequence.
Conclusions: Our data demonstrate that highly zoonotic cervid prion strains are not only possible but also can be stably maintained in cervids and that CWD zoonosis is prion strain-dependent.
Funded by: NIH
Grant number: R01NS052319, R01NS088604, R01NS109532
Acknowledgement: We want to thank the National Prion Disease Pathology Surveillance Center and Drs. Allen Jenny and Katherine O’Rourke for providing the sCJD samples and the CWD samples, respectively.
Adaptation of chronic wasting disease (CWD) prion strains in hosts with different PRNP genotypes
Camilo Duque Velasqueza,c, Elizabeth Triscotta,c, Chiye Kima,c, Diana Morenoa,c, Judd Aikenb,c, and Debbie McKenziea,c
aDepartment of Biological Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; bDepartment of Agriculture, Food & Nutritional Science, University of Alberta, Edmonton, AB T6G 2G8, Canada; cCentre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, AB T6G 2M8, Canada
Aims: The contagious nature of CWD epizootics and the PrPC amino acid variation of cervids (and susceptible sympatric species) guarantee the expansion of prion conformational diversity and selective landscapes where new strains can arise. CWD strains can have novel transmission properties including altered host range that may increase zoonotic risk as circulating strains diversify and evolve. We are characterizing the host adaptability of characterized CWD strains as well as CWD isolates from different cervid species in various enzootic regions.
Material and Methods: Characterized CWD strains as well as a number of isolates from hunter-harvested deer were bioassayed in our rodent panel (transgenic mice expressing cervid alleles G96, S96 and H95-PrPC, elk PrPC, bovine PrPC, and both hamsters and non-transgenic laboratory mice). Strain characteristics were compared using computer based scoring of brain pathology (e.g. PrPCWD brain distribution), western blot and protein misfolding cyclic amplification (PMCA).
Results: Transmission of various isolates resulted in the selection of strain mixtures in hosts expressing similar PrPC, particularly for polymorphic white-tailed deer and for Norwegian reindeer. As of the second passage, transmission of P153 moose prions from Norway has not resulted in emergence of strains with properties similar to any North American CWD strains in our taxonomic collection (Wisc-1, CWD2, H95+and 116AG).
Conclusions: Our data indicates polymorphic white-tailed deer can favor infection with more than one strain. Similar to transmission studies of Colorado CWD isolates from cervids expressing a single PrPC primary structure, the isolate from Norway reindeer (V214) represents a strain mixture, suggesting intrinsic strain diversity in the Nordfjella epizootic. The diversity of CWD strains with distinct transmission characteristics represents a threat to wildlife, sympatric domestic animals and public health.
Funded by: Genome Canada and Genome Alberta (Alberta Prion Research Institute and Alberta Agriculture & Forestry); NSERC Grant number: #LSARP 10205; NSERC RGPIN-2017-05539
Acknowledgement: We would like to thank Margo Pybus (Alberta Environment and Parks) Trent Bollinger (University of Saskatchewan) for providing us with tissue samples from hunter-harvested deer and Sylvie Benestad for providing moose and reindeer samples.
Application of PMCA to understand CWD prion strains, species barrier and zoonotic potential
Sandra Pritzkowa, Damian Gorskia, Frank Ramireza, Fei Wanga, Glenn C. Tellingb, Justin J. Greenleec, Sylvie L. Benestadd, and Claudio Sotoa aDepartment of Neurology, University of Texas Medical School at Houston, Houston, Texas, USA; bDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA; cVirus and Prion Research Unit, United States Department of Agriculture, Ames, Iowa, USA; dNorwegian Veterinary Institute, OIE Reference Laboratory for CWD, Ås, Norway
Aims: Chronic wasting disease (CWD) is a prion disease affecting various species of cervids that continues to spread uncontrollably across North America and has recently been detected in Scandinavia (Norway, Sweden and Finland). The mechanisms responsible for the natural transmission of CWD are largely unknown. Furthermore, the risk of CWD transmission to other species, including humans, is also unknown and remains a dangerous enigma. In this study, we investigated the potential of CWD prions to infect several other animal species (sheep, cattle, pig, hamster, and mouse) including humans, by examining their capacity to convert the normal prion protein of distinct species in a PMCA reaction. Moreover, we also investigated whether the in vivo passage of CWD through intermediate species alters their capacity for zoonotic transmission, which may represent a major hazard to human health.
Material and Methods: For these studies, we used brain material from CWD-infected white-tailed deer (Odocoileus virginianus), elk (Cervus canadensis), and mule deer (Odocoileus hemionus) as species native to North America. We also used CWD-infected Moose (Alces alces), reindeer (Rangifer tarandus) and red deer (Cervus elaphus) as Norwegian cervids. We also used brains from cattle, sheep and pigs experimentally infected by CWD. To study interspecies-transmission and zoonotic potential, samples were tested via PMCA for the conversion of PrPCinto PrPScusing different combinations of inoculum and host species. Based on these analyses we estimated the spillover and zoonotic potential for different CWD isolates. We define and quantify spillover and zoonotic potential indices as the efficiency by which CWD prions sustain prion generation in vitro at the expense of normal prion proteins from various mammals and human, respectively.
Results: Our results show that prions from some cervid species, especially those found in Northern Europe, have a higher potential to transmit disease characteristics to other animals. Conversely, CWD-infected cervids originated in North America appear to have a greater potential to generate human PrPSc. We also found that in vivo transmission of CWD to cattle, but not to sheep or pigs substantially increases the ability of these prions to convert human PrPCby PMCA.
Conclusions: Our findings support the existence of different CWD prion strains with distinct spillover and zoonotic potentials. We also conclude that transmission of CWD to other animal species may increase the risk for CWD transmission to humans. Our studies may provide a tool to predict the array of animal species that a given CWD prion could affect and may contribute to understanding the risk of CWD for human health.
Funded by: National Institute of Health Grant number: P01 AI077774
Generation of human chronic wasting disease in transgenic mice
Zerui Wanga, Kefeng Qinb, Manuel V. Camachoa, Ignazio Cali a,c, Jue Yuana, Pingping Shena, Tricia Gillilanda, Syed Zahid Ali Shaha, Maria Gerasimenkoa, Michelle Tanga, Sarada Rajamanickama, Anika Yadatia, Lawrence B. Schonbergerd, Justin Greenleee, Qingzhong Konga,c, James A. Mastriannib, and Wen-Quan Zoua,c
aDepartment of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA; bDepartment of Neurology and Center for Comprehensive Care and Research on Memory Disorders, the University of Chicago Pritzker School of Medicine, Chicago, USA; cNational Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA; dDivision of High-Consequence Pathogens and Pathology, Centers for Disease Control and Prevention, 1600 Clifton Rd, Atlanta, GA, USA; eVirus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Avenue, Ames, IA, USA
Aims: Chronic wasting disease (CWD) results from the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC) in the brains of deer and elk. It has been spreading rapidly throughout many regions of North America, exported inadvertently to South Korea, and more recently identified in Europe. Mad cow disease has caused variant Creutzfeldt-Jakob disease (vCJD) in humans and is currently the only known zoonotic prion disease. Whether CWD is transmissible to humans remains uncertain. The aims of our study were not only to confirm whether CWD prion isolates can convert human brain PrPC into PrPSc in vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPSc is infectious.
Material and Methods: Eight CWD prion isolates from 7 elks and 1 deer were used as the seeds while normal human brain homogenates containing either PrP-129 MM (n = 2) or PrP-129 VV (n = 1) were used as the substrates for sPMCA assay. A normal elk brain tissue sample was used as a negative control seed. Two lines of humanized transgenic (Tg) mice expressing either human PrP-129VV or −129 MM polymorphism were included for transmission studies to determine the infectivity of PMCA-amplified PrPSc. Wester blotting and immunohistochemistry and hematoxylin & eosin staining were used for determining PrPSc and neuropathological changes of inoculated animals.
Results: We report here the generation of the first CWD-derived infectious human PrPSc using elk CWD PrPSc to initiate conversion of human PrPC from normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPSc was derived from the human brain PrPC substrate. Two lines of humanized transgenic mice expressing human PrPC with either Val or Met at the polymorphic codon 129 developed clinical prion disease following intracerebral inoculation with the PMCA-generated CWD-derived human PrPSc. Diseased mice exhibited distinct PrPSc patterns and neuropathological changes in the brain.
Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSc has the potential to overcome the species barrier and directly convert human PrPC into infectious PrPSc that can produce bona fide prion disease when inoculated into humanized transgenic mice.
Funded by: CJD Foundation and NIH
Mortality surveillance of persons potentially exposed to chronic wasting disease
R.A. Maddoxa, R.F. Klosb, L.R. Willb, S.N. Gibbons-Burgenerb, A. Mvilongoa, J.Y. Abramsa, B.S. Applebyc, L.B. Schonbergera, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bWisconsin Department of Health Services (WDHS), Division of Public Health, Madison, USA; cNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA
Aims: It is unknown whether chronic wasting disease (CWD), a prion disease of cervids, can infect people, but consumption of meat from infected animals would be the most likely route of transmission. Wisconsin Department of Health Services, Division of Public Health (WDHS) personnel maintain a database consisting of information collected from hunters who reported eating, or an intention to eat, venison from CWD-positive cervids. These data, collected since 2003, allow for the evaluation of causes of mortality in individuals potentially exposed to CWD.
Material and Methods: The WDHS database contains the name, date of birth, when available, year of CWD-positive deer harvest, and city and state of residence for each potentially exposed individual. The database also includes information on how the deer was processed (self-processed or by a commercial operator) and when applicable, names of others with whom the venison was shared. Duplicate entries (i.e., those who consumed venison from CWD-positive deer in multiple hunt years) are determined by first name, last name, and date of birth. All names in the database are cross-checked with reported cases of human prion disease in Wisconsin and cases in the National Prion Disease Pathology Surveillance Center (NPDPSC) diagnostic testing database. Persons with date of birth available are also cross-checked with prion disease decedents identified through restricted-use national multiple cause-of-death data via a data use agreement with the National Center for Health Statistics (NCHS).
Results: The database currently consists of 1561 records for hunt years 2003–2017 and 87 additional records for 2018–2019. Of these, 657 records have accompanying date of birth; 15 entries were removed as duplicates leaving 642 unique individuals. Of these individuals, 278 of 426 (66%) who ate venison from a CWD-positive deer and provided processing information reported self-processing. No matches were found among any persons in the database cross-checked with WDHS human prion disease surveillance data, NPDPSC data (February 2022 update), and NCHS data through 2020.
Conclusions: Because of the linkage of person and CWD-positive animal in the WDHS database, reviewing the cause of mortality in potentially exposed persons is possible. The number of individuals cross-checked so far is likely only a small percentage of those potentially exposed to CWD in Wisconsin, and many more years of vital status tracking are needed given an expected long incubation period should transmission to humans occur. Nevertheless, the findings of this ongoing review are thus far reassuring.
Prion disease incidence, United States, 2003–2020
R.A. Maddoxa, M.K. Persona, K. Kotobellib, A. Mvilongoa, B.S. Applebyb, L.B. Schonbergera, T.A. Hammetta, J.Y. Abramsa, and E.D. Belaya aNational Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), Atlanta, USA; bNational Prion Disease Pathology Surveillance Center (NPDPSC), Case Western Reserve University, Cleveland, USA
Aims: Mortality data, in conjunction with neuropathological and genetic testing results, are used to estimate prion disease incidence in the United States.
Material and Methods: Prion disease decedents for 2003–2020 were identified from restricted-use U.S. national multiple cause-of-death data, via a data use agreement with the National Center for Health Statistics, and from the National Prion Disease Pathology Surveillance Center (NPDPSC) database. NPDPSC decedents with neuropathological or genetic test results positive for prion disease for whom no likely match was found in the NCHS multiple cause-of-death data were added as cases for incidence calculations, while those with negative neuropathology results but with cause-of-death data indicating prion disease were removed. Unmatched cases in the NPDPSC database lacking neuropathological testing but with a positive real-time quaking-induced conversion (RT-QuIC) test result were additionally assessed. Age-specific and age-adjusted average annual incidence rates were calculated from the combined data; the year 2000 as the standard population and the direct method were used for age-adjustment.
Results: A total of 7,921 decedents were identified as having prion disease during 2003–2020 for an age-adjusted average annual incidence of 1.2 per million population. The age-adjusted incidence between males and females (1.3 and 1.1 per million, respectively) differed significantly (p < 0.0001). The age-specific average annual incidence among those <55 and ≥55 years of age was 0.2 and 4.8 per million, respectively; incidence among those ≥65 was 6.1 per million. Eighteen cases were <30 years of age for an age-specific incidence of 8.0 per billion; only 6 of these very young cases were sporadic (3 sporadic CJD, 3 sporadic fatal insomnia), with the rest being familial (9), variant (2), or iatrogenic (1). The age-adjusted annual incidence for the most recent year of data, 2020, was 1.3 per million. However, assessment of RT-QuIC positive cases lacking neuropathology in the NPDPSC database suggested that approximately 20% more cases may have occurred in that year; the addition of a subset of these cases that had date of death information available (n = 44) increased the 2020 rate to 1.4 per million.
Conclusions: Mortality data supplemented with the results of neuropathological, CSF RT-QuIC, and genetic testing can be used to estimate prion disease incidence. However, the identification in the NPDPSC database of RT-QuIC-positive cases lacking date of death information suggests that this strategy may exclude a number of probable prion disease cases. Prion disease cases <30 years of age, especially those lacking a pathogenic mutation, continue to be very rare.
Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer
Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera
aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK
Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.
Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.
Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.
Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.
Funded by: National Institutes of Health (NIH)
Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM
Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies
Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study
Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa
aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.
Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.
Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.
Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.
Funded by: USDA
Grant number: AP20VSSPRS00C143
ATYPRION project: assessing the zoonotic potential of interspecies transmission of CWD isolates to livestock (preliminary results).
Enric Vidala,b, Juan Carlos Espinosac, Samanta Gilera,b, Montserrat Ordóñeza,b, Guillermo Canteroa,b, Vincent Béringued, Justin J. Greenleee, and Juan Maria Torresc
aUnitat mixta d’Investigació IRTA-UAB en Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; bIRTA. Programa de Sanitat Animal. Centre de Recerca en Sanitat Animal (CReSA). Campus de la Universitat Autònoma de Barcelona (UAB), Bellaterra, Catalonia; cCentro de Investigación en Sanidad Animal, CISA-INIA-CSIC, Valdeolmos, Madrid, Spain; dMolecular Virology and Immunology, French National Research Institute for Agriculture, Food and Environment (INRAE), Université Paris-Saclay, Jouy-en-Josas, France; eVirus and Prion Research Unit, National Animal Disease Center, ARS, United States Department of Agriculture, Ames, IA, USA
Aims: Since variant Creutzfeldt-Jackob disease was linked to the consumption of bovine spongiform encephalopathy prions, the study of the pathobiological features of animal prions, particularly their zoonotic potential, is of great concern to the scientific community and public health authorities. Furthermore, interspecies transmission of prions has been demonstrated as a putative evolutionary mechanism for prions, that can lead to the emergence of new features including the ability to infect humans. For instance, small ruminants’ atypical scrapie prions, when propagated in a bovine or porcine host, can shift to a classical BSE phenotype thus posing a potential risk in case of human exposure. So far, no hard evidence of zoonotic transmission of cervids’ chronic wasting disease (CWD) to humans has been published, however experimental transmission to bovine, ovine and caprine hosts has been achieved. Our goal is to investigate if, once passaged through these domestic species, CWD prions might become infectious to humans.
Material and Methods: Different CWD isolates experimentally adapted to cattle, sheep and goat (Hamir et al, 2005, 2006, 2007, Greenlee et al 2012) have been intracerebrally inoculated to transgenic mouse models expressing the human cellular prion protein either homozygous for methionine or valine at codon 129 (Tg340-Met129 and Tg362-Val129). Additionally, inocula obtained from experimental transmission of elk CWD to ovinized (Tg501) and bovinized (BoTg110) transgenic mice, as well as white-tailed deer CWD to BoTg110 mice, are currently being bioassayed in both human PrPC transgenic models.
Results and conclusions: No evidence of transmission has been found on first passage for bovine adapted elk and mule deer CWD to none of the humanized models. The remaining bioassays are ongoing without showing clinical signs yet, as well as second passages for the negative 1stpassages.
Funded by: La Marató de TV3 foundation. Grant number: ATYPRION (201,821–30-31-32)
Prion Conference 2018 Abstracts
P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States
Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)
(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.
Background
Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.
Methods
Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).
Results
Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).
Conclusions
While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.
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P172 Peripheral Neuropathy in Patients with Prion Disease
Wang H(1), Cohen M(1), Appleby BS(1,2)
(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.
Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein can also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.
We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.
Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.
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P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission
Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)
(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.
Background
Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.
Methods
We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.
Results
We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.
Conclusions
PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.
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P180 Clinico-pathological analysis of human prion diseases in a brain bank series
Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)
(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.
Background and objective:
The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.
Methods:
We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.
Results:
176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to Gerstmann Sträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.
Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.
Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.
Discussion:
A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:
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P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures
Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)
(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.
Aims:
Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.
Methods:
Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.
Results:
The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.
Conclusions:
Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.
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WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice
Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)
(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.
To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.
See also poster P103
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.
=====
WA16 Monitoring Potential CWD Transmission to Humans
Belay ED
Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.
The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.
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P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan
Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)
(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.
Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.
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Source Prion Conference 2018 Abstracts
Volume 24, Number 8—August 2018
Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions
Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)
Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.
snip...
Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).
A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.
The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.
In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).
The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.
Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.
Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.
This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.
Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.
Prion 2017 Conference Abstracts
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1
University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen
This is a progress report of a project which started in 2009.
21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.
Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.
At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE
8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.
SATURDAY, FEBRUARY 23, 2019
Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019
TUESDAY, NOVEMBER 04, 2014
Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011
Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "
Transmission Studies
Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS
resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.
snip....
Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿
Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations
In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.
TUESDAY, MAY 11, 2021
***> A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet <***
Conclusion
We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.
Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.
''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''
Singeltary submission to the BSE Inquiry on CJD and Nutritional Supplements 1998
ABOUT that deer antler spray and CWD TSE PRION...
I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease.
just saying...
I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.
Sender: "Patricia Cantos"
To: "Terry S Singeltary Sr. (E-mail)"
Subject: Your submission to the Inquiry
Date: Fri, 3 Jul 1998 10:10:05 +0100
3 July 1998
Mr Terry S Singeltary Sr.
E-Mail: Flounder at wt.net
Ref: E2979
Dear Mr Singeltary,
Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments.
Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.
As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.
Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;
http://www.bse.org.uk.
Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it?
In the meantime, thank you for you comments. Please do not hesitate to contact me on...
snip...end...tss
everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year _previously_ and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. _both_ cases confirmed. ...kind regards, terry
TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS
IPLEX, mad by standard process;
vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach.
also;
what about potential mad cow candy bars ?
see their potential mad cow candy bar list too...
THESE are just a few of MANY of just this ONE COMPANY...TSS
DEPARTMENT OF HEALTH AND HUMAN SERVICES
FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE
Friday, January 19, 2001 snip...
17 But I think that we could exhibit some quite
18 reasonable concern about blood donors who are taking dietary
19 supplements that contain a certain amount of unspecified-
20 origin brain, brain-related, brain and pituitary material.
21 If they have done this for more than a sniff or something
22 like that, then, perhaps, they should be deferred as blood
23 donors.
24 That is probably worse than spending six months in
25 the U.K.
1/19/01
3681t2.rtf(845) page 501
see full text ;
My neighbors Mom also died from CJD.
She had been taking a nutritional supplement which contained the following;
vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver
powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried
porcine stomach. As I said, this woman taking these nutritional
supplements, died from CJD.
BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999) Cite this as: BMJ 1999;319:1312
Singeltary Submission recorded here;
SUNDAY, NOVEMBER 10, 2013
LARGE CJD TSE PRION POTENTIAL CASE STUDY AMONG HUMANS WHO TAKE DEER ANTLER VELVET WILL BE ONGOING FOR YEARS IF NOT DECADES, but who's cares $
SUNDAY, JULY 24, 2016
Chronic Wasting Disease Prions in Elk Antler Velvet and Marketing of this Product in Nutritional Supplements for Humans?
Saturday, May 1, 2021
***> Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease
ENVIRONMENTAL FACTORS FOR CHRONIC WASTING DISEASE CWD TSE PRION
October 6th-12th, 126th Meeting 2022 Resolutions
RESOLUTION NUMBER: 30 Approved
SOURCE: COMMITTEE ON WILDLIFE
SUBJECT MATTER: Chronic Wasting Disease Carcass Disposal Dumpster Management and Biosecurity
BACKGROUND INFORMATION:
State and tribal wildlife agencies may identify collection points (dumpsters) within an identified chronic wasting disease (CWD) management zone for the disposal of hunter-harvested cervid carcasses to remove potentially infected carcasses off the landscape for disposal by an approved method (Gillin & Mawdsley, 2018, chap.14). However, depending on their placement and maintenance these dumpsters could potentially increase the risk of CWD transmission.
In several different states, photographic evidence has shown dumpsters in state identified CWD management zones overflowing with deer carcasses and limbs scattered on the land nearby. This could provide an opportunity for scavengers to potentially move infected carcass material to non-infected zones or increase contamination of the ground material around the dumpster’s location.
Federal guidance does not explicitly address uniform standards for collection locations for carcasses of free-ranging cervids; however, the United States Department of Agriculture, Animal and Plant Health Inspection Service, Veterinary Services Program Standards on CWD outlines procedures for carcass disposal, equipment sanitation, and decontamination of premises for captive cervid facilities.
RESOLUTION:
The United States Animal Health Association urges the Association of Fish and Wildlife Agencies (AFWA), Wildlife Health Committee to further refine the AFWA Technical Report on Best Management Practices for Prevention, Surveillance, and Management of Chronic Wasting Disease; Chapter 14, Carcass Disposal to address the placement and management of chronic wasting disease carcass disposal dumpsters or other carcass collection containers.
Reference:
1. Gillin, Colin M., and Mawdsley, Jonathan R. (eds.). 2018. AFWA Technical Report on Best Management Practices for Surveillance, Management and Control of Chronic Wasting Disease. Association of Fish and Wildlife Agencies, Washington, D. C. 111 pp.
PRION CONFERENCE 2022 ABSTRACTS CWD TSE PrP ZOONOSIS and ENVIRONMENTAL FACTORS
Chronic wasting disease detection in environmental and biological samples from a taxidermy site
Paulina Sotoa,b, J. Hunter Reedc, Mitch Lockwoodc, and Rodrigo Moralesa,b aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bUniversidad Bernardo O’Higgins, Santiago, Chile; cTexas Parks and Wildlife Department, Texas, USA
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy affecting captive and free-ranging cervids (e.g., mule deer, white-tailed deer, elk, reindeer, and moose). Nowadays, CWD is widely distributed in North America. It is suggested that CWD spreads due to direct animal contact or through exposure to contaminated environments previously inhabited by infected animals. CWD may also be spread through the movement of infected animals and carcasses. Taxidermy practices involve processing deer tissues (or whole animal carcasses). In many cases, the CWD status of processed animals is unknown. This can generate risks of disease spread and transmission. Taxidermy practices include different steps involving physical, chemical, and biological procedures. Without proper tissue handling or disposal practices, taxidermist facilities may become a focus of prion infectivity.
Aims: In this study, we evaluated the presence of infectious prions in a taxidermy facility believed to be exposed to CWD. Detection was performed using the Protein Misfolding Cyclic Amplification (PMCA) technique in biological and inert environmental samples. Methods: We collected biological and environmental samples (plants, soils, insects, excreta, and others) from a taxidermy facility, and we tested these samples using the PMCA technique. In addition, we swabbed different surfaces possibly exposed to CWD-infected animals. For the PMCA reaction, we directly used a swab piece or 10 µL of 20% w/v homogenized samples.
Results: The PMCA analysis demonstrated CWD seeding activity in some of the components of this facility, including insects involved in head processing, soils, and a trash dumpster.
Conclusions: Different areas of this property were used for various taxidermy procedures. We were able to detect the presence of prions in
i) soils that were in contact with the heads of dead animals,
ii) insects involved in the cleaning of skulls, and
iii) an empty dumpster where animal carcasses were previously placed.
This is the first report demonstrating that swabbing is a helpful method to screen for prion infectivity on surfaces potentially contaminated with CWD. These findings are relevant as this swabbing and amplification strategy may be used to evaluate the disease status of other free-ranging and captive settings where there is a concern for CWD transmissions, such as at feeders and water troughs with CWD-exposed properties. This approach could have substantial implications for free-ranging cervid surveillance as well as in epidemiological investigations of CWD.
ENVIRONMENT FACTORS FOR THE TRANSMISSION OF CWD TSE PRP
Sensitive detection of chronic wasting disease prions recovered from environmentally relevant surfaces
Environment International
Available online 13 June 2022, 107347
Environment International
Sensitive detection of chronic wasting disease prions recovered from environmentally relevant surfaces
Qi Yuana Gag e Rowdenb Tiffany M.Wolfc Marc D.Schwabenlanderb Peter A.LarsenbShannon L.Bartelt-Huntd Jason C.Bartza
a Department of Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska, 68178, United States of America
b Department of Veterinary and Biomedical Sciences, University of Minnesota, Saint Paul, MN, 55108, United States of America
c Department of Veterinary Population Medicine, University of Minnesota, Saint Paul, MN, 55108, United States of America
d Department of Civil and Environmental Engineering, Peter Kiewit Institute, University of Nebraska-Lincoln, Omaha, Nebraska, 68182, United States of America
Received 26 April 2022, Revised 8 June 2022, Accepted 9 June 2022, Available online 13 June 2022.
Get rights and content
Under a Creative Commons license Open access
Highlights • An innovative method for prion recovery from swabs was developed.
• Recovery of prions decreased as swab-drying time was increased.
• Recovery of CWD prions from stainless steel and glass was approximately 30%.
• RT-QuIC enhanced CWD prion detection by 4 orders of magnitude.
• Surface-recovered CWD prion was sufficient for efficient RT-QuIC detection.
Abstract
Chronic wasting disease (CWD) has been identified in 30 states in the United States, four provinces in Canada, and recently emerged in Scandinavia. The association of CWD prions with environmental materials such as soil, plants, and surfaces may enhance the persistence of CWD prion infectivity in the environment exacerbating disease transmission. Identifying and quantifying CWD prions in the environment is significant for prion monitoring and disease transmission control. A systematic method for CWD prion quantification from associated environmental materials, however, does not exist. In this study, we developed an innovative method for extracting prions from swabs and recovering CWD prions swabbed from different types of surfaces including glass, stainless steel, and wood. We found that samples dried on swabs were unfavorable for prion extraction, with the greatest prion recovery from wet swabs. Using this swabbing technique, the recovery of CWD prions dried to glass or stainless steel was approximately 30% in most cases, whereas that from wood was undetectable by conventional prion immunodetection techniques. Real-time quake-induced conversion (RT-QuIC) analysis of these same samples resulted in an increase of the detection limit of CWD prions from stainless steel by 4 orders of magnitude. More importantly, the RT-QuIC detection of CWD prions recovered from stainless steel surfaces using this method was similar to the original CWD prion load applied to the surface. This combined surface swabbing and RT-QuIC detection method provides an ultrasensitive means for prion detection across many settings and applications.
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5. Conclusions
Chronic wasting disease is spreading in North America and it is hypothesized that in CWD-endemic areas environmental persistence of CWD prions can exacerbate disease transmission. The development of a sensitive CWD prion detection method from environmentally relevant surfaces is significant for monitoring, risk assessment, and control of CWD. In this study, we developed a novel swab-extraction procedure for field deployable sampling of CWD prions from stainless steel, glass, and wood. We found that extended swab-drying was unfavorable for extraction, indicating that hydrated storage of swabs after sampling aided in prion recovery. Recoverable CWD prions from stainless steel and glass was approximately 30%, which was greater than from wood. RT-QuIC analysis of the swab extracts resulted in an increase of the detection limit of CWD prions from stainless steel by 4 orders of magnitude compared to conventional immunodetection techniques. More importantly, the RT-QuIC detection of CWD prions recovered from stainless steel surfaces using this developed method was similar to the original CWD prion load without surface contact. This method of prion sampling and recovery, in combination with ultrasensitive detection methods, allows for prion detection from contaminated environmental surfaces.
Research Paper
Cellular prion protein distribution in the vomeronasal organ, parotid, and scent glands of white-tailed deer and mule deer
Anthony Ness, Aradhana Jacob, Kelsey Saboraki, Alicia Otero, Danielle Gushue, Diana Martinez Moreno, Melanie de Peña, Xinli Tang, Judd Aiken, Susan Lingle & Debbie McKenzie ORCID Icon show less
Pages 40-57 | Received 03 Feb 2022, Accepted 13 May 2022, Published online: 29 May 2022
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ABSTRACT
Chronic wasting disease (CWD) is a contagious and fatal transmissible spongiform encephalopathy affecting species of the cervidae family. CWD has an expanding geographic range and complex, poorly understood transmission mechanics. CWD is disproportionately prevalent in wild male mule deer and male white-tailed deer. Sex and species influences on CWD prevalence have been hypothesized to be related to animal behaviours that involve deer facial and body exocrine glands. Understanding CWD transmission potential requires a foundational knowledge of the cellular prion protein (PrPC) in glands associated with cervid behaviours. In this study, we characterized the presence and distribution of PrPC in six integumentary and two non-integumentary tissues of hunter-harvested mule deer (Odocoileus hemionus) and white-tailed deer (O. virginianus). We report that white-tailed deer expressed significantly more PrPC than their mule deer in the parotid, metatarsal, and interdigital glands. Females expressed more PrPC than males in the forehead and preorbital glands. The distribution of PrPC within the integumentary exocrine glands of the face and legs were localized to glandular cells, hair follicles, epidermis, and immune cell infiltrates. All tissues examined expressed sufficient quantities of PrPC to serve as possible sites of prion initial infection, propagation, and shedding.
KEYWORDS: Prion chronic wasting diseasesex differences species differences disease prevalence cervid protein expression glands
Paper
Rapid recontamination of a farm building occurs after attempted prion removal
Kevin Christopher Gough BSc (Hons), PhD Claire Alison Baker BSc (Hons) Steve Hawkins MIBiol Hugh Simmons BVSc, MRCVS, MBA, MA Timm Konold DrMedVet, PhD, MRCVS … See all authors
First published: 19 January 2019 https://doi.org/10.1136/vr.105054
The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.
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This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.
***>This is very likely to have parallels with control efforts for CWD in cervids.
***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years
***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded.
JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12
Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free
Gudmundur Georgsson1, Sigurdur Sigurdarson2, Paul Brown3
Front. Vet. Sci., 14 September 2015 | https://doi.org/10.3389/fvets.2015.00032
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
imageTimm Konold1*, imageStephen A. C. Hawkins2, imageLisa C. Thurston3, imageBen C. Maddison4, imageKevin C. Gough5, imageAnthony Duarte1 and imageHugh A. Simmons1
The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.
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Discussion
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In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination.
***> 172. Establishment of PrPCWD extraction and detection methods in the farm soil
Kyung Je Park, Hoo Chang Park, In Soon Roh, Hyo Jin Kim, Hae-Eun Kang and Hyun Joo Sohn
Foreign Animal Disease Division, Animal and Plant Quarantine Agency, Gimcheon, Gyeongsangbuk-do, Korea
Conclusions: Our studies showed that PrPCWD persist in 0.001% CWD contaminated soil for at least 4 year and natural CWD-affected farm soil. When cervid reintroduced into CWD outbreak farm, the strict decontamination procedures of the infectious agent should be performed in the environment of CWD-affected cervid habitat.
THE tse prion aka mad cow type disease is not your normal pathogen.
The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.
you cannot cook the TSE prion disease out of meat.
you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.
the TSE prion agent also survives Simulated Wastewater Treatment Processes.
IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.
you can bury it and it will not go away.
The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.
it’s not your ordinary pathogen you can just cook it out and be done with.
***> that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.
1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8
***> Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of
Neurological Disorders and Stroke, National Institutes of Health,
Bethesda, MD 20892.
Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
PMID: 8006664 [PubMed - indexed for MEDLINE]
New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production
MONDAY, APRIL 19, 2021
Evaluation of the application for new alternative biodiesel production process for rendered fat including Category 1 animal by-products (BDI-RepCat® process, AT) ???
Detection of protease-resistant cervid prion protein in water from a CWD-endemic area
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing
Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals
THURSDAY, FEBRUARY 28, 2019
BSE infectivity survives burial for five years with only limited spread
5 or 6 years quarantine is NOT LONG ENOUGH FOR CWD TSE PRION !!!
QUARANTINE NEEDS TO BE 21 YEARS FOR CWD TSE PRION !
FRIDAY, APRIL 30, 2021
Should Property Evaluations Contain Scrapie, CWD, TSE PRION Environmental Contamination of the land?
***> Confidential!!!!
***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!
---end personal email---end...tss
and so it seems...
Scrapie Agent (Strain 263K) Can Transmit Disease via the Oral Route after Persistence in Soil over Years
Published: May 9, 2007
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Our results showed that 263K scrapie agent can persist in soil at least over 29 months. Strikingly, not only the contaminated soil itself retained high levels of infectivity, as evidenced by oral administration to Syrian hamsters, but also feeding of aqueous soil extracts was able to induce disease in the reporter animals. We could also demonstrate that PrPSc in soil, extracted after 21 months, provides a catalytically active seed in the protein misfolding cyclic amplification (PMCA) reaction. PMCA opens therefore a perspective for considerably improving the detectability of prions in soil samples from the field.
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Dr. Paul Brown Scrapie Soil Test BSE Inquiry Document
Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer
Nathaniel D. Denkersa, Erin E. McNultya, Caitlyn N. Krafta, Amy V. Nallsa, Joseph A. Westricha, Wilfred Goldmannb, Candace K. Mathiasona, and Edward A. Hoovera
aPrion Research Center, College of Veterinary Medicine and Biological Sciences, Department of Microbiology, Immunology, and Pathology; Colorado State University, Fort Collins, CO, USA; bDivision of Infection and Immunity, The Roslin Institute and the Royal Dick School of Veterinary Studies, University of Edinburgh, Midlothian, UK
Aims: Chronic wasting disease (CWD) now infects cervids in South Korea, North America, and Scandinavia. CWD is unique in its efficient transmission and shedding of prions in body fluids throughout long course infections. Questions remain as to the magnitude of shedding and the route of prion acquisition. As CWD continues to expand, the need to better understand these facets of disease becomes more pertinent. The purpose of the studies described was to define the longitudinal shedding profile of CWD prions in urine, saliva, and feces throughout the course of infection in white-tailed deer.
Material and Methods: Twelve (12) white-tailed deer were inoculated with either 1 mg or 300ng of CWD. Urine, saliva, and feces were collected every 3-month post-inoculation (MPI) throughout the study duration. Cohorts were established based on PNRP genotype: codon 96 GG (n = 6) and alternate codons 96 GS (n = 5) & 103NT (n = 1). Urine and saliva were analyzed using iron-oxide magnetic extraction (IOME) and real-time quaking induced conversion (RT-QuIC)(IQ). Feces were subjected to IOME, followed by 4 rounds protein misfolding cyclic amplification (PMCA) with products analyzed by RT-QuIC (IPQ). To determine whether IPQ may be superior to IQ, a subset of urine and saliva were also tested by IPQ. Results were compared with clinical disease status.
Results: Within the 96 GG cohort, positive seeding activity was detected in feces from all deer (100%), in saliva from 5 of 6 (83%), and in urine from 4 of 6 (66%). Shedding in all excreta occurred at, or just after, the first positive tonsil biopsy result. In the 96 GS/103NT cohort, positive seeding activity could be detected in feces from 3 of 6 (50%) deer, saliva in 2 of 6 (33%), and urine in 1 of 6 (16%). Shedding in excreta was detected >5 months after the first tonsil positive result. Four of six 96 GG deer developed clinical signs of CWD, whereas only 2 of the 96 GS/103NT did. Shedding was more frequently detected in deer with clinical disease. The IPQ protocol did not significantly improve detection in saliva or urine samples, however, it significantly augmented detection in feces by eliminating non-specific background commonly experienced with IQ. Negative control samples remained negative in samples tested.
Conclusions: These studies demonstrate: (a) CWD prion excretion occurs throughout infection; (2) PRNP genotype (GG≫GS/NT) influences the excreta shedding; and (3) detection sensitivity in excreta can vary with different RT-QuIC protocols. These results provide a more complete perspective of prion shedding in deer during the course of CWD infection.
Funded by: National Institutes of Health (NIH)
Grant number: RO1-NS061902-09 R to EAH, PO1-AI077774 to EAH, and R01-AI112956-06 to CKM
Acknowledgement: We abundantly thank Sallie Dahmes at WASCO and David Osborn and Gino D’Angelo at the University of Georgia Warnell School of Forestry and Natural Resources for their long-standing support of this work through provision of the hand-raised, CWD-free, white-tailed deer used in these studies
Large-scale PMCA screening of retropharyngeal lymph nodes and in white-tailed deer and comparisons with ELISA and IHC: the Texas CWD study
Rebeca Benaventea, Paulina Sotoa, Mitch Lockwoodb, and Rodrigo Moralesa
aDepartment of Neurology, McGovern Medical School, University of Texas Health Science Center at Houston, Texas, USA; bTexas Park and Wildlife Department, Texas, USA
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that affects various species of cervids, and both free-ranging and captive animals. Until now, CWD has been detected in 3 continents: North America, Europe, and Asia. CWD prevalence in some states may reach 30% of total animals. In Texas, the first case of CWD was reported in a free-range mule deer in Hudspeth and now it has been detected in additional 14 counties. Currently, the gold standard techniques used for CWD screening and detection are ELISA and immunohistochemistry (IHC) of obex and retropharyngeal lymph nodes (RPLN). Unfortunately, these methods are known for having a low diagnostic sensitivity. Hence, many CWD-infected animals at pre-symptomatic stages may be misdiagnosed. Two promising in vitro prion amplification techniques, including the real-time quaking-induced conversion (RT-QuIC) and the protein misfolding cyclic amplification (PMCA) have been used to diagnose CWD and other prion diseases in several tissues and bodily fluids. Considering the low cost and speed of RT-QuIC, two recent studies have communicated the potential of this technique to diagnose CWD prions in RPLN samples. Unfortunately, the data presented in these articles suggest that identification of CWD positive samples is comparable to the currently used ELISA and IHC protocols. Similar studies using the PMCA technique have not been reported.
Aims: Compare the CWD diagnostic potential of PMCA with ELISA and IHC in RPLN samples from captive and free-range white-tailed deer. Material and Methods: In this study we analyzed 1,003 RPLN from both free-ranging and captive white-tailed deer collected in Texas. Samples were interrogated with the PMCA technique for their content of CWD prions. PMCA data was compared with the results obtained through currently approved techniques.
Results: Our results show a 15-fold increase in CWD detection in free-range deer compared with ELISA. Our results unveil the presence of prion infected animals in Texas counties with no previous history of CWD. In the case of captive deer, we detected a 16% more CWD positive animals when compared with IHC. Interestingly, some of these positive samples displayed differences in their electroforetic mobilities, suggesting the presence of different prion strains within the State of Texas.
Conclusions: PMCA sensitivity is significantly higher than the current gold standards techniques IHC and ELISA and would be a good tool for rapid CWD screening.
Funded by: USDA
Grant number: AP20VSSPRS00C143
Prion 2022 Conference abstracts: pushing the boundaries
Published: 06 September 2021
***> Chronic wasting disease: a cervid prion infection looming to spillover
Alicia Otero, Camilo Duque Velásquez, Judd Aiken & Debbie McKenzie
Veterinary Research volume 52, Article number: 115 (2021)
CHRONIC WASTING DISEASE CASESCWD STATUS OF CAPTIVE HERDS Updated January 2023
Date of Index Case Confirmation Index Case State County Species Herd Type HCP Enrolled HCP Certified Number of Animals Herd Status
Terry S. Singeltary Sr.
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