Chronic Wasting Disease: Michigan Chronic Wasting Disease CWD TSE Prion 2022 Update

Michigan Chronic Wasting Disease CWD TSE Prion 2022 Update

CWD Testing Results for Deer Harvested in 2021

Statewide Total 7229

25 positive

https://www.michigan.gov/dnr/managing-resources/wildlife/cwd/hunters/cwd-testing-results-for-deer-harvested-in-2021

Western Upper Peninsula Citizen Advisory Council

DNR Division Reports

Date of Production: January 2021 (Information current as of 12/30/2020)

This documentation is provided by Michigan DNR staff as a supplement for Western UPCAC Council members. Please email or call with any questions.

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CWD

▪ The DNR has tested 2020 deer statewide this year for CWD.

the U.P. 333 deer have been tested for CWD from the Core Surveillance Area with the majority of those coming from Dickinson county.

No additional positive deer have been detected to date in the UP samples and 17 positive animals have been detected in the lower peninsula this year with 198 total positives since testing began in 2015.

https://www.michigan.gov/dnr/-/media/Project/Websites/dnr/Documents/Boards/WUPCAC/WUPCAC-Division-Reports/report-jan-2021.pdf

''with 198 total positives since testing began in 2015.''

APPENDIX A: 2020 REPORTABLE DISEASES

Livestock Diseases:

Disease Species Number of Animals

CWD (Chronic Wasting Disease) Cervid 46

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SCRAPIE

Scrapie

Metric 2019 2020

Compliance actions for inadequate official identification observed at livestock markets 51 30

Scrapie identification compliance at livestock markets for sheep 98% 96%

Scrapie identification compliance at livestock markets for goats 94% 93%

https://www.michigan.gov/mdard/-/media/Project/Websites/mdard/documents/annual-reports/aid/2020_aid_annual_report.pdf

''CWD (Chronic Wasting Disease) Cervid 46''

APPENDIX A: 2021 REPORTABLE DISEASES

Livestock Diseases:

Disease Species Number of Animals

CWD (Chronic Wasting Disease) Cervid 19

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SCRAPIE

Metric 2020 2021

Compliance actions for inadequate official identification observed at livestock markets 30 35

Scrapie identification compliance at livestock markets for sheep 96% 99%

Scrapie identification compliance at livestock markets for goats 93% 86%

https://www.michigan.gov/mdard/-/media/Project/Websites/mdard/documents/annual-reports/aid/2021_aid_annual_report.pdf

''CWD (Chronic Wasting Disease) Cervid 19''

Michigan CWD TSE Prion 2022 Hunting Digest

Where has chronic wasting disease (CWD) been found in Michigan? Since the initial finding of CWD on May 20, 2015, free-ranging deer in Clinton, Dickinson, Eaton, Gratiot, Ingham, Ionia, Isabella, Jackson, Kent and Montcalm counties have been positively confirmed with CWD. Please visit Michigan.gov/CWD for more information on CWD and the latest news and testing updates. See pages 56-57 and 61-62 for important regulations pertaining to CWD.

see cwd positive maps for Michigan on pages starting at around page 31. seems difficult for Michigan et al to give an exact cwd positive count an any given time.

https://www.michigan.gov/dnr/-/media/Project/Websites/dnr/Documents/LED/digests/hunting_digest.pdf

Michigan Natural Resources Commission Update CWD TSE Prion April 14, 2022

https://www.michigan.gov/dnr/-/media/Project/Websites/dnr/Documents/Boards/NRC/2022/April-2022/April_14_2022_Presentations.pdf

Special Surveillance Area (SSA) Counties for Farmed Deer

https://www.michigan.gov/mdard/-/media/Project/Websites/mdard/documents/animals/cervids/special_surveillance_areas_for_farmed_deer.pdf

Assessing drivers of spread and transmission of Chronic Wasting Disease in Michigan deer

Primary Contact: Dr. Dwayne Etter, DNR Wildlife Division, Lansing, Michigan Email: etterd@michigan.gov Phone: (517) 284-4720 DNR Financial Support: $120,149 in FY19, $502,737 total. Study Area: South-central Lower Peninsula Time Frame: 10/1/2017-9/30/2022

Abstract:

The occurrence of chronic wasting disease (CWD) in Michigan challenges the foundations of wildlife conservation, both in the short term and perhaps more significantly in the longer term. In the short term, CWD is causing reallocation of precious financial and staff-time resources and will be widely disruptive to existing programs of the Michigan Department of Natural Resources (DNR). In the longer term, diseases such as CWD pose a threat to the financial cornerstone of fisheries and wildlife programs because sales of deer hunting licenses represent a large proportion of annual revenue for the Division of Wildlife. Recognizing these threats, the Division of Wildlife included wildlife disease in its Guiding Principles and Strategies (Objective 1.3: monitor and preserve the health of Michigan’s wildlife) and prepared a comprehensive Surveillance and Response Plan for Chronic Wasting Disease of Free-ranging and Privately Owned Cervids.

Chronic wasting disease is a transmissible spongiform encephalopathy that infects North American cervids including white-tailed deer (Williams 2005). The infectious agent of CWD is a misfolded protein, a prion, which accumulates in the brainstem and lymphatic tissue of infected animals and results in neurodegeneration and eventual death. In states where CWD is established it has emerged as a major threat, reducing the health of populations and causing long-term population decline (Edmunds et al. 2016, Gross and Miller 2001, Manjerovic et al. 2014).

The discovery of CWD in Michigan creates an immediate need for population monitoring and surveillance of at-risk deer populations. Since 2015, nine infected individuals have been identified following collection through state surveillance efforts, representing key successes in targeted disease management. However, the continued discovery of infected individuals in 2016 suggests a high likelihood that additional infected individuals remain on the landscape. The occurrence of a small number of infected animals across a relatively small geographic region in mid-Michigan indicates that the disease is still emerging.

What distinguishes the research proposed here from extensive work done in other states is that CWD is still in an emergent phase in Michigan. Michigan discovered the disease early during a time when transmission of the disease may be more dependent on the density of deer on the landscape because most infections are through direct contact of infected animals with susceptible individuals. This situation is similar only to New York and Minnesota. In all other states where CWD has been discovered, the disease was already well established, and transmission included infection mediated by contact of susceptible individuals with severely contaminated environments. Our research in Michigan is intended to explore management options for the

12 IC4117 (Rev. 02/02/2021)

control of an emerging occurrence of CWD through better understanding of behavior and population dynamics of deer inhabiting areas of known infection.

Our objectives address the strategic plans set forth by the Michigan DNR to “1.3.1: Develop and implement strategies to prevent and control diseases before they occur; 1.3.2: Respond to wildlife disease outbreaks; 1.3.4: Conduct research and monitoring to provide information to make management recommendations regarding wildlife disease; 1.3.5: Raise awareness regarding current and emerging wildlife health issues; and 1.3.6: Work with State and Federal agencies, and stakeholders to address wildlife health issues.”

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Management of Chronic Wasting Disease in Michigan

Primary Contact: Dr. Kelly Straka, DNR Wildlife Division, Lansing, Michigan Email: StrakaK1@michigan.gov Phone: (517) 336-5030 DNR Financial Support: $50,000 in FY19, $250,000 total. Study Area: Statewide. Time Frame: 10/01/2016-09/30/2022

Abstract: Chronic Wasting Disease (CWD) is a transmissible spongiform encephalopathy that infects North American cervids including white-tailed deer (Williams 2005). The infectious agent of CWD is a misfolded protein, a prion, that accumulates in the brainstem and lymphatic tissue of infected animals and results in neurodegeneration and eventual death. In states where CWD is established, it has emerged as a major threat, reducing the health of populations and causing long-term population decline (Edmunds et. al. 2016, Gross and Miller 2001, Manjerovic et. al. 2014).

15 IC4117 (Rev. 02/02/2021)

The occurrence of CWD in Michigan challenges the foundations of wildlife conservation, both in the short term and perhaps more significantly in the longer term. In the short term, CWD is causing reallocation of precious financial and staff-time resources and will be widely disruptive to existing programs. In the longer term, diseases such as CWD pose a threat to the financial cornerstone of fisheries and wildlife programs because sales of deer hunting licenses represent such a large proportion of annual revenue. Recognizing these threats, the Wildlife Division included wildlife disease in its Guiding Principles and Strategies (Objective 1.3: Monitor and preserve the health of Michigan’s wildlife) and prepared a comprehensive Surveillance and Response Plan for Chronic Wasting Disease of Free-ranging and Privately Owned Cervids.

The discovery of CWD in Michigan creates an immediate need for tools that better assess the return-oninvestment of funds for surveillance and management of CWD. We propose building on risk assessment and modeling that was previously developed during a CWD outbreak in New York. There, we showed how costs of CWD containment could be reduced dramatically by using risk modeling procedures and mapping areas where management action would have the greatest impact on disease control (Williams et. al. 2014). We plan to expand on those efforts by adapting them to Michigan and drawing on newly emerging tools for population estimation and risk analysis procedures that we have been using on other research (e.g., local-scale monitoring of deer populations using distance sampling and evaluation of wild turkey harvest regulations using statistical risk modeling).

Our objectives address the strategies set forth in the Wildlife Division’s Guiding Principles and Strategies to “1.3.1: Develop and implement strategies to prevent and control diseases before they occur, 1.3.2: Respond to wildlife disease outbreaks, 1.3.4: Conduct research and monitoring to provide information to make management recommendations regarding wildlife disease, 1.3.5: Raise awareness regarding current and emerging wildlife health issues and 1.3.6: Work with State and Federal agencies and stakeholders to address wildlife health issues.” Specifically, we will provide managers with decision tools to: (1) evaluate the risk of spread of disease against the geographic extent of management action and attendant financial and political costs, (2) evaluate management alternatives to control CWD and assess the risk of local cases of CWD transitioning from emergent status to established status (where the disease becomes a self-sustaining reservoir within a population) and (3) monitor management outcomes for deer population abundance and disease prevalence.

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Quantifying Upper Peninsula deer movements and abundance: preparing for CWD management

Primary Contact: Dr. Dean Beyer Jr., DNR Wildlife Division, Marquette, Michigan Email: beyerd@michigan.gov Phone: (906) 228-6561 DNR Financial Support: $117,759 in FY19, $613,001 total. Study Area: Upper Peninsula Time Frame: 10/1/2017-9/30/2021

Abstract:

Chronic wasting disease (CWD) occurs in free-ranging white-tailed deer in Lower Michigan, and in our neighboring state of Wisconsin where the disease is endemic. Although wildlife managers have not documented CWD in the Upper Peninsula, managers found infected deer in two Wisconsin captive cervid facilities near the Michigan border. Officials identified the disease in a facility in Oneida County, Wisconsin, about 40 km from our Iron County border and a second positive deer in Oconto County, Wisconsin, about 50 km from our Menominee County border.

While it is not possible to predict if or when we will find CWD in the Upper Peninsula, preparations seem prudent. A scientifically based understanding of deer movements and estimates of population abundance are critical for developing management recommendations in response to CWD. Deer movements and abundance can influence the probability of disease occurrence, contact rates which can affect transmission rate, and geographic extent of an outbreak (e.g., Oyer et al. 2007, Skult et al. 2008, Webb et al. 2010). Importantly, these data take time to gather and managers need this information at the time of first discovery. Thus, waiting for a disease outbreak before gathering these data would put managers at a disadvantage. Important deer movements to understand include seasonal home ranges, migration (especially important in the Upper Peninsula), dispersal, transient, and exploratory.

Information on these movements would inform decisions on identification of CWD management zones. The current strategy is to establish a 16-km radius circle around the location of an infected cervid and include entire counties whose boundaries intersect this circle as part of the CWD management zone. Further, if results from local population surveys or other credible scientific data suggest that cervids from within the radius are likely to move beyond the management zone boundary, the boundary should be expanded accordingly. In the Upper Peninsula, deer can seasonally migrate 50 km (Van Deelen et al. 1998), with overall movements exceeding 80 km (Doepker et al. 2015). These migratory movements, as well as other movements (e.g., dispersal), are currently unknown and certainly not aligned with or contained within county boundaries. Although some information exists on deer movements in the UP, most of this work relied on tag returns that do not provide the needed level of spatial and temporal resolution to inform management responses to a disease outbreak.

The Upper Peninsula Region (UPR), Biological and Social Sciences Section (BSSS), Wildlife Health Section (WHS), and Mississippi State University (MSU) wish to develop a program to address the need for information on deer movements. The core work would entail deploying GPS collars on deer in select wintering complexes and conditional winter range (starting along WI border) and documenting movements over three years. To complete the capture and collaring work, we would work cooperatively with interested sportspersons.

https://www.michigan.gov/dnr/-/media/Project/Websites/dnr/Documents/FRD/Mgt/IC4117_SustainableForestryResearchSumm.pdf

Michigan MDARD: Chronic Wasting Disease Confirmed in a Farmed White-Tailed Deer from Mecosta County

For immediate release: May 9, 2022 Media contact: Chelsea Lewis, 517-331-1151 or Jennifer Holton, 517-284-5724

MDARD: Chronic Wasting Disease Confirmed in a Farmed White-Tailed Deer from Mecosta County

LANSING, MI – Today, the Michigan Department of Agriculture and Rural Development (MDARD) confirmed chronic wasting disease (CWD) in one white-tailed deer from a farmed cervid facility in Mecosta County. The infected three-year-old deer was discovered through routine testing as part of the state’s CWD surveillance program for farmed deer.

CWD is a fatal neurological disease that affects different cervid species, including white-tailed deer, mule deer, elk, and moose. The disease can be transmitted directly from one animal to another and indirectly through the environment. While an infected animal may appear healthy for months or years, it will eventually display abnormal behavior, progressive weight loss, and physical debilitation in the later stages of the disease.

“MDARD is committed to limiting the spread and impact of this disease. CWD surveillance testing plays an integral part in accomplishing this goal because it helps us to detect and respond to the disease promptly,” said State Veterinarian Dr. Nora Wineland. “In addition, our continued partnership with farmed cervid owners, hunters, and other state and federal partners is also essential to ensure the health of Michigan’s farmed deer population.”

The presence of CWD in farmed cervid facilities and free-ranging deer is not new to Michigan. Since 2008, including this new case, CWD has been detected at 10 Michigan cervid farms in the following counties: Kent (2), Mecosta (4), Montcalm (3), and Newaygo.

With free-ranging deer, CWD was first discovered in May 2015; and cases have been found across nine counties in Michigan’s Upper and Lower Peninsulas. To date, no free-ranging white-tailed deer have tested positive for CWD in Mecosta County.

As part of MDARD’s disease response, investigations are ongoing to rule out any possible exposure to other farmed cervids.

Currently, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend that CWD-infected animals should not be consumed as food by either humans or domestic animals.

More information about CWD can be found at Michigan.gov/CWD or Michigan.gov/MDARD-Cervid.

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https://www.michigan.gov/mdard/about/media/pressreleases/2022/05/09/cwd-confirmed-in-a-farmed-white-tailed-deer-from-mecosta-county

Michigan’s 2021 deer seasons included targeted CWD surveillance, 25 positive deer

April 14, 2022

Hunters encouraged to share harvest results via online survey

Buck walking through lush green forest

Though Michigan’s 2021 deer hunting seasons ended in late January 2022, the Michigan Department of Natural Resources is continuing to accept feedback from hunters about their experiences. Hunter harvest surveys have been sent to a random sample of the state’s deer hunters. In addition, hunters can take a brief online survey. Final harvest survey results will be presented later this summer.

Initial data from Michigan’s 2021 deer hunting seasons – including chronic wasting disease testing results and deer license sales information – was presented at Thursday’s meeting of the Michigan Natural Resources Commission in Lansing, with highlights shared below.

A shift in CWD testing

The DNR has finalized its 2021 surveillance efforts for chronic wasting disease, ultimately testing just over 7,200 deer. The more targeted testing goals are part of the department’s new region-by-region strategy aimed at detecting new outbreaks rather than revisiting known ones.

“We want to thank hunters for their cooperation in helping us meet our CWD surveillance goals,” said DNR Director Dan Eichinger. “Strategic testing for chronic wasting disease is of primary importance for the department, and we couldn’t meet these goals without the committed assistance of deer hunters.”

Eichinger also praised the work of deer processors, taxidermists and local businesses that help collect samples for testing, and other key partners who provide necessary assistance to the department.

In all, 25 CWD-positive deer were confirmed in 2021. Three cases of CWD were detected in Isabella County, which represents a new county where the disease has been found. (Since Michigan’s first confirmation of a CWD-positive wild deer in 2015, CWD has been detected in white-tailed deer in Clinton, Dickinson, Eaton, Gratiot, Ingham, Ionia, Jackson, Kent and Montcalm counties.)

Doe walking through late summer forest “It was not unexpected to find positive cases in Isabella County, as these detections were fairly close to where we’ve identified cases in Montcalm and northern Gratiot County,” said DNR deer and elk specialist Chad Stewart. “Our main areas of infection remain in parts of Montcalm and northeast Kent counties, as well as southern Jackson County, where we knew CWD existed going into the 2021 hunting season.”

Despite the department’s finding of 25 positive animals last year, Stewart cautioned against comparing the low number of positives with the high number of deer tested and concluding there is not a problem.

“The distribution of our samples greatly affects the number of positives we expect to find. Intensive collection of samples in known CWD locations like Montcalm and Kent counties would certainly lead to a high number of positives being detected,” he said. “Our goal this year was to begin to understand what CWD looks like in areas that are historically under-sampled, and we made a lot of strides on that front.”

Stewart said that chronic wasting disease is going to be a problem for parts of Michigan’s deer herd in the future: “Once it becomes established, it is unlikely that we can reverse course on the disease. Prevention and early detection remain our best options for CWD management.”

Hunter walking across plain while sun shines brightly CWD surveillance moving forward

For Michigan’s 2021 deer seasons, the DNR started a multiyear process of strategic, focused CWD surveillance in regions around the state. Last year’s surveillance occurred mainly in the three tiers of counties near the Ohio border. Over the next few years, the remainder of the state will be systematically sampled to determine if CWD is present in other areas where it hasn’t yet been identified.

Hunter numbers

While there was a temporary rise in hunter numbers during the COVID-19 pandemic, participation is declining in Michigan. The trend is not new, nor is it only being observed here. States across the country are feeling the financial pressure of reduced hunter numbers, because sales of hunting licenses comprise a large portion of the funding for critical conservation work.

“Nationwide, hunting has seen a gradual decline over the last several decades,” said Eichinger. “The trend is likely due to a combination of factors including generations of hunters who are aging out of the sport, and younger generations that are less likely to participate in hunting due to societal changes and more competition for their attention.”

Deer hunter numbers in 2021 were down nearly 4% over the previous year with close to 600,000 hunters purchasing a deer license. Hunter number declines are in line with past years going back to peak participation in the mid 90’s.

“While the trend in hunter participation is discouraging, we know that hunting remains an important part of Michigan’s outdoor heritage,” Eichinger said. “That’s why we encourage experienced hunters to introduce the sport to new hunters wherever they can. Spending time with veteran hunters can reduce the learning curve, increase safety and instill a sense of excitement and appreciation for our state’s natural resources.”

To learn more about deer management, CWD and deer hunting in Michigan, and to access the 2021 deer harvest survey, visitMichigan.gov/Deer.

https://www.michigan.gov/dnr/about/newsroom/releases/2022/04/14/2021-deer-seasons-included-targeted-cwd-surveillance

Michigan Chronic Wasting Disease CWD TSE Prion Update

CWD in Michigan

Since May 2015 when the first CWD deer was found in Michigan, CWD has been confirmed in a number of townships in the Lower Peninsula. As of October 2018, a CWD positive deer was found in the Upper Peninsula in Dickinson County. CWD was also found in August 2008 at a Kent County deer farm facility and in January 2017 in two captive deer that were from a deer farm facility in Mecosta County.

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516---,00.html

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90541---,00.html

CWD TESTING RESULTS

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90536-538324--,00.html

Desperado Deer: The Persistent Problem of Captive Deer Running Wild

by Editor | May 8, 2018 | Conservation, Hunting

Cervid Escapees – Measuring the Problem

For starters, no one knows for sure how many deer escape from high-fence facilities each year. Neither the DNR nor the Michigan Department of Agriculture and Rural Development keep accurate, complete records of the number of escapees reported by citizens and/or investigated by DNR conservation officers. Only in 2017 did the DNR first begin using an electronic database to monitor escaped cervids.

Currently, there are 333 licensed captive cervid facilities in the state, most of which are breeding farms (161) or hunting ranches (132), holding over 21,000 whitetail deer, fallow deer, red deer, Sitka deer and elk. While the number of deer escapees voluntarily reported has declined over recent years, the numbers do not include escapees that are never reported.

https://www.michiganoutofdoors.com/desperado-deer-the-persistent-problem-of-captive-deer-running-wild/

Chronic wasting disease (CWD) is a transmissible neurological disease found in deer and elk populations that produces small lesions in brains of infected animals. As a result, CWD causes weight loss and a decline in body control. It is a species-specific disease, and there have been no cases in humans or other animals.

Currently, to determine the presence of CWD, brain and lymph node samples are taken by an accredited veterinarian after an animal dies. These samples are then submitted for testing.

As part of their operations, all privately-owned cervid (POC) facilities in Michigan are required to submit samples. The number of samples that must be submitted depends on what specific program that a producer participates in: the Chronic Wasting Disease Herd Certification Program (CWD HCP) or the Surveillance Program.

First, for the CWD HCP, all cervids 12 months of age and older that die for any reason must be tested for CWD.

Second, all facilities that are not a part of the CWD HCP must participate in the Surveillance Program. The Surveillance Program requires that all animals 12 months of age and older that die from illness, injury, or euthanasia due to disease must be tested for CWD. In addition, 25% of cervids slaughtered, hunted, or culled must be tested. This number is calculated on an annual basis. In general, all facilities that have at least one death must test at least this one animal.

Samples for either of these programs can be submitted to a private veterinarian, the Michigan State University Veterinary Diagnostic Lab, or an MDARD drop off location.

For more information, contact the MDARD Cervid Program.

General Questions/Concerns: MDARD-Cervid@Michigan.gov

Cervid Program Manager:

Dr. Jennifer Calogero CalogeroJ@Michigan.gov 517-284-5692

Cervid Program Secretary: Melanie Hart HartM1@Michigan.gov 517-284-5679

https://www.michigan.gov/mdard/animals/cervids/chronic-wasting-disease-cwd-surveillance

Privately Owned Cervidae

Raising deer and elk in captivity is jointly regulated by the DNR and the Michigan Department of Agriculture and Rural Development. The DNR oversees the registration of facilities containing farmed cervids and performs inspections of these operations. MDARD manages the disease programs for the state’s POC facilities. Participation in disease surveillance programs - such as those for chronic wasting disease (CWD) and the bovine tuberculosis (TB) - ensures for a robust industry by increasing the marketability of these animals by decreasing their potential for carrying disease. There are nearly 300 licensed facilities in 76 Michigan counties totaling over 63,000 fenced acres. The division conducts about 95 facility inspections per year to ensure that fencing and recordkeeping meet industry standards.

2021 Wildlife Health Section Accomplishments

Tested over 8,000 deer heads for bovine tuberculosis and

2,500 heads for chronic wasting disease.

While CWD is not known to be zoonotic, bTB can infect humans, domestic animals and wild animals beyond white-tailed deer.

https://storymaps.arcgis.com/stories/79560ac896024e6cb8d925831904a5c7

***> Tested ...and 2,500 heads for chronic wasting disease.

MDNR estimates put the Michigan deer population around 1.75 million for 2019. Dec 25, 2019

Issues Pros and Cons Despite federal, state, and local regulations and other measures intended to prevent the spread or reduce CWD prevalence, the disease continues to be identified in captive cervid facilities certified as “low risk” through the United States Department of Agriculture (USDA) Herd Certification Program and the CFIA (Canadian Food Inspection Agency) Voluntary Herd Certification Programs (participating in a federally-approved CWD program was a measure of the ATA program). According to the USDA data reports, there were 22 new CWD-positive captive cervid facilities identified in FY2020; 41 percent of those were either enrolled or certified in the federal HCP program. There are a variety of unregulated processes used to collect urine, and they often result in the accumulation of a mixture of secretions, therefore providing concurrent contaminated risks. In addition, urine products are frequently batched/combined from multiple locations and distributed across the country, which increases the likelihood of CWDinfected urine entering the market. There are currently no standard regulations to ensure that urine collected for lures and attractants are disease-free.

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Biological

Nationally, CWD continues to be found in captive cervid facilities.

From the years 2012 to 2021, there have been 66 privately owned cervid facilities nationally where CWD has been identified.

Of those 66 facilities, 39 were enrolled in the HCP, and 32 of those facilities were HCP-certified (meaning there had been at least five years of disease monitoring and no rule violations) indicating low risk for CWD.

This national USDA CWD HCP is not mandatory, and more importantly, recent CWD events show that it does not and cannot guarantee that captive deer herds are CWD free.

To date, CWD has been found in more than 140 captive deer herds in 16 states and two Canadian provinces.

https://www.michigan.gov/documents/dnr/Signed_05WCO2021_724156_7.pdf

Front. Vet. Sci., 18 January 2022 | https://doi.org/10.3389/fvets.2021.824815

Evaluation of Real-Time Quaking-Induced Conversion, ELISA, and Immunohistochemistry for Chronic Wasting Disease Diagnosis

All, except one, CWD positive RLNs analyzed were from ten Counties geographically located in the West Michigan region of the Lower Peninsula. Taken together, we show evidence that the RT-QuIC assay is comparable to ELISA and IHC and could be helpful for routine CWD detection in surveillance programs. RT-QuIC also demonstrated that CWD prions are distributed across lymph nodes in a variety of anatomic locations.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8803730/pdf/fvets-08-824815.pdf

MICHIGAN CWD CAPTIVE

11/4/2021 2, 3 Y Male MI Kent Elk Breeder Yes Yes 0 Depopulated

7/15/2021 4 Y Female MI Montcalm WTD Breeder No No 109 Quarantine

4/18/2021 2.5 Y Male MI WTD Shooter No No ukn Quarantine

3/3/2021 4 Y Male MI Montcalm WTD Shooter No NA 14 Quarantine

12/2019 3, 4.5 Y Males MI Newaygo WTD Shooter No No >600 Quarantine

4/2019 2.5 Y Female MI Montcalm WTD Breeder No NA 113 Depopulated

12/2017 1.5 Y Female MI Mecosta WTD Breeder Yes Yes 525 Quarantined

1/2017 2Y Female MI Mecosta WTD & Sika deer Shooter No NA 71 Depopulated

https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf

Michigan:

September, 2019: NVSL confirmed CWD in a two year old female white-tailed deer in Montcalm County. The doe was a natural addition to the breeding herd which consists of 50 white-tailed deer. This herd is not enrolled in the Federal HCP, is within a CWDendemic area, and is under quarantine.

https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/usaha-annual-cervid-health-report-2019.pdf

Michigan: One new CWD positive herd

Hunt preserve of >600 WTD, not in HCP, populated and under quarantine

https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/voluntary-cwd-hcp-annual-update-fy2020

Farmed Cervid Chronic Wasting Disease Management and Response Activities 2021 Cooperative Agreements

https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/cwd-funding-oppt-annc-fy2021.pdf

https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervid-cwd-2021-funding-faqs

APHIS also conducts monitoring and surveillance activities to detect diseases that affect cervids, including chronic wasting disease (CWD) and tuberculosis (TB). APHIS’ voluntary national CWD Herd Certification Plan (HCP) works with States, Tribes, and the cervid industry to control CWD in farmed cervids by allowing the interstate movement only from certified herds.

Currently, 28 States participate in the national CWD HCP. In FY 2019 APHIS tested more than 11,000 farmed cervids for CWD.

As a result, APHIS identified 17 new CWD positive farmed cervid herds.

https://www.usda.gov/sites/default/files/documents/20aphis2021notes.pdf ;

Wild Cervid Chronic Wasting Disease Management and Response Activities 2021 Funding Opportunity

USDA APHIS Wildlife Services announced awards for two opportunities for the control and prevention of CWD in wild cervids under the titles of “Wild Cervid CWD Management and Response Activities 2021” and “Tribal Nations Wild Cervid CWD Opportunities 2021.” See information below.
Wild Cervid CWD FOA
Wild Cervid CWD FAQs
Wild Cervid CWD Management and Response Activities 2021 Cooperative Agreements
Wild Cervid CWD 2021 Project Executive Summaries

Tribal Nations Wild Cervid Chronic Wasting Disease Opportunities 2021 Funding Opportunity

VS Farmed Cervid Chronic Wasting Disease Management and Response Activities 2021 Funding Opportunity

RISK, UNCERTAINTY AND DECISION-MAKING: ASSESSING CHRONIC WASTING DISEASE OCCURRENCE RISK ACROSS AN EMERGENCE SPECTRUM

Exposure hazards included point locations of captive cervid facilities, deer processors and taxidermists, and out-of-area hunting connectivity.

As of 2018, there were a total of 296 ranch and full-registration facilities in Michigan. In terms of their spatial distribution, there were facilities in 196 out of 1240 townships, and 71 out of 83 counties (Fig. 2.1). There were 468 registered deer processors and taxidermists in 2017, the year for which data were available. Processors and taxidermists occurred in 696 out of 1240 townships, and 82 out of 83 counties (Fig. 2.2). Lastly, out-of-area hunting was based on both intrastate and interstate metrics. For intrastate hunting, the percentage of respondents that travelled from one county to another was scaled up to the total number of hunters that reside in a county. CWD positive counties included Clinton, Dickinson, Eaton, Gratiot, Ingham, Ionia, Jackson, Kent and Montcalm counties. The average number of hunters per resident county from 2013 – 2017 who travelled to a CWD positive county ranged from 0 – 3832 per year (Fig. 2.3). A low number (i.e., low connectivity) of interstate hunters ranged from 0 – 1059 for the 5-year average, whereas a high number ranged from 1060 – 3832 (Fig. 2.3). Interstate hunting was quantified as the number of Michigan resident hunters who purchased an out-of-state license in Wisconsin between years 2013 – 2017 (i.e., nonresident license). For interstate connectivity, I found that average annual number of Michigan county residents that traveled to Wisconsin between 2013 –2017 was 0 – 170 per year (Fig. 2.4). A low number (i.e., low connectivity) of interstate hunters ranged from 0 – 39 for the 5-year average, whereas a high number ranged from 40 – 170 (Fig. 2.4).

snip...

In 2017, the Michigan Department of Natural Resources (MDNR) detected CWD in a 3- year-old white-tailed doe submitted during an early season youth hunt (MDNR 2017). Additional surveillance in the area during 2017 identified 45 total CWD-positive animals in a concentrated disease focus in the west-central Lower Peninsula of Michigan within Kent and Montcalm counties. Nine previous CWD detections had occurred in the state in 2015 and 2016; however, the 2017 detections were the first evidence that CWD might be widespread and established within Michigan. Based on a single year of observation, predicting the area affected by the cluster of disease with distance benchmarks would likely fail to fully encapsulate the affected area. Furthermore, based on the sparsity of data, fitting complex disease models was not possible. Thus, there was an immediate need for an alternative approach that could more appropriately estimate the extent of CWD and identify locations at high risk using limited available information

https://d.lib.msu.edu/etd/49428/datastream/OBJ/View/

Prion protein polymorphisms in Michigan white-tailed deer (Odocoileus virginianus)

Caitlin N. Ott-ConnORCID Icon,Julie A. Blanchong &Wes A. Larson

Pages 183-190 | Received 22 Jul 2021, Accepted 01 Oct 2021, Published online: 09 Nov 2021

Download citation https://doi.org/10.1080/19336896.2021.1990628 ;

ABSTRACT

Chronic Wasting Disease (CWD), a well-described transmissible spongiform encephalopathy of the Cervidae family, is associated with the aggregation of an abnormal isoform (PrPCWD) of the naturally occurring host prion protein (PrPC). Variations in the PrP gene (PRNP) have been associated with CWD rate of infection and disease progression. We analysed 568 free-ranging white-tailed deer (Odocoileus virginianus) from 9 CWD-positive Michigan counties for PRNP polymorphisms. Sampling included 185 CWD-positive, 332 CWD non-detected, and an additional 51 CWD non-detected paired to CWD-positives by sex, age, and harvest location. We found 12 polymorphic sites of which 5 were non-synonymous and resulted in a change in amino acid composition. Thirteen haplotypes were predicted, of which 11 have previously been described. Using logistic regression, consistent with other studies, we found haplotypes C (OR = 0.488, 95% CI = 0.321–0.730, P < 0.001) and F (OR = 0.122, 95% CI = 0.007–0.612, P < 0.05) and diplotype BC (OR = 0.340, 95% CI = 0.154–0.709, P < 0.01) were less likely to be found in deer infected with CWD. As has also been documented in other studies, the presence of a serine at amino acid 96 was less likely to be found in deer infected with CWD (P < 0.001, OR = 0.360 and 95% CI = 0.227–0.556). Identification of PRNP polymorphisms associated with reduced vulnerability to CWD in Michigan deer and their spatial distribution can help managers design surveillance programmes and identify and prioritize areas for CWD management.

snip...

Results

PRNP sequences were determined for 568 free-ranging white-tailed deer from 9 CWD-positive Michigan counties. Of these samples, 185 were CWD-positive, 332 were CWD non-detected, and an additional 51 CWD non-detected were paired to CWD-positives to control for sex, age, and harvest location (Figure 1). Within the analysed 625bp region of the PRNP gene, we detected 12 single nucleotide polymorphisms (SNPs), 9 of which had been previously reported [22, 29, 33, 36, 38–41]. Of the 12 SNPs, 5 were non-synonymous, resulting in a change to the amino acid sequence (Table 1). BLAST and literature searches indicated that 589A/G, 642 G/A, and 643 C/A had not previously been reported. Full associated sequences have been deposited in GenBank under accession numbers MZ913400 – MZ913401.

snip...

As CWD detections continue to increase the areas under surveillance, the use of regionally specific data to allocate testing efforts and funding will be pivotal for success. Identification of PRNP polymorphisms associated with reduced vulnerability to CWD and their spatial distribution and prevalence may help managers design surveillance programmes to identify and prioritize areas for CWD management when partnered with movement data and anticipated deposition of prions onto the landscape over time.

https://www.tandfonline.com/doi/full/10.1080/19336896.2021.1990628

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.

https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197

Special Surveillance Area (SSA) Counties for Farmed Deer

Calhoun (CA)

Clinton (CN)

Dickinson (DK)

Eaton (ET)

Gratiot (GT)

Hillsdale (HD)

Ingham (IN)

Ionia (IO)

Jackson (JK)

Kent (KN)

Lenawee (LN)

Livingston (LV)

Mecosta (MT)

Menominee (MO)

Montcalm (MT)

Newaygo (NW)

Saginaw (SG)

Shiawassee (SH)

Washtenaw (WA)

SSAs as of December 18, 2019

SSAs are established when a free-ranging or farmed deer is identified with chronic wasting disease.

Contact the Michigan Department of Agriculture and Rural Development’s Cervid Program at MDARD-Cervid@Michigan.gov or 517-284-5679.

https://www.michigan.gov/mdard/-/media/Project/Websites/mdard/documents/animals/cervids/special_surveillance_areas_for_farmed_deer.pdf?rev=6497528a63b94975a982c9fc08039db3&hash=4AA0BEE0C1C96A0D34EE935AA0F55189

Expanding Distribution of Chronic Wasting Disease ACTIVE

By National Wildlife Health Center February 5, 2022

https://www.usgs.gov/centers/nwhc/science/expanding-distribution-chronic-wasting-disease

Michigan CWD Testing Results Deer Harvested in 2021 Statewide Total 22 Positive To Date For Year in Wild

CWD Testing Results for Deer Harvested in 2021

Test results updated as of December 13, 2021.

Totals reflected in this update only include those with final test results.

Deer with pending results are not included in these totals.

Zone Total Tested Number Positive

UP CWD Core Surveillance Area 193 0

South Isabella + Gratiot 749 3

South Jackson 855 12

Totals 1797 15

Testing numbers above are part of the county totals in the larger table below.

County Name Total Tested Number Positive

Allegan 290 0

Barry 150 0

Berrien 77 0

Branch 104 0

Calhoun 139 0

Cass 64 0

Eaton 82 0

Hillsdale 204 0

N. Jackson 135 0

Kalamazoo 196 0

Lenawee 118 0

Livingston 68 0

Macomb 13 0

Monroe 34 0

Oakland 43 0

St. Joseph 81 0

Van Buren 155 0

Washtenaw 178 0

Wayne 9 0

Total to date 2106 0

These counties are open for hunter service testing November 15-18 ONLY. There are no surveillance goals.

County Name Total Tested Number Positive

Clinton 45 0

Dickinson (non-core) 3 0

Ingham 32 0

Ionia 35 1

Kent 47 1

Montcalm 53 5

Total to date 215 7 Deer tested in remainder of state 113 0 Positive

Statewide Total 3865 22 Positive

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90536-538324--,00.html

Michigan MDARD Chronic Wasting Disease Confirmed in Two Farmed Elk from Kent County

For immediate release: November 18, 2021 Media contact: Chelsea Lewis-Parisio, 517-331-1151

MDARD: Chronic Wasting Disease Confirmed in Two Farmed Elk from Kent County

LANSING, MI – Today, the Michigan Department of Agriculture and Rural Development (MDARD) confirmed two cases of chronic wasting disease (CWD) in elk from a farmed cervid facility in Kent County. The two infected elk, a two-and-a-half-year-old and a three-and-a-half-year-old, were discovered through disease tracing efforts that resulted from finding CWD in a different Michigan farmed cervid herd. These are the first cases of CWD in Michigan elk.

“The discovery of chronic wasting disease in elk housed at a facility linked to a positive animal is not surprising,” said State Veterinarian Dr. Nora Wineland, DVM. “MDARD’s main priority is to limit the spread of this disease by working together with other state departments, farmers, and ranchers. These findings underscore how important it is to pay attention to CWD and the movement of animals that may allow the disease to spread.”

The presence of CWD in farmed cervid facilities and free-ranging deer is not new to Michigan. Since 2008, including these new cases, CWD has been detected at nine Michigan cervid farms in the following counties: Kent (2), Mecosta (3), Montcalm (3), and Newaygo.

No wild elk have tested positive for CWD in Michigan. The disease was first discovered in free-ranging deer in May 2015; cases have been found across nine counties in Michigan’s Upper and Lower Peninsulas. To date, 37 free-ranging white-tailed deer have tested positive for CWD in Kent County.

As part of MDARD’s disease response, investigations are ongoing to rule out any possible exposure to other farmed cervids.

Currently, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend that animals that have tested positive for CWD should not be consumed as food by either humans or domestic animals.

More information about CWD can be found at http://Michigan.gov/CWD or http://Michigan.gov/MDARD-Cervid.

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https://content.govdelivery.com/accounts/MIDARD/bulletins/2fcd9a1

THURSDAY, APRIL 14, 2022

Michigan’s 2021 deer seasons included targeted CWD surveillance, 25 positive deer

https://chronic-wasting-disease.blogspot.com/2022/04/michigans-2021-deer-seasons-included.html

WEDNESDAY, NOVEMBER 10, 2021

Prion protein polymorphisms in Michigan white-tailed deer (Odocoileus virginianus)

https://chronic-wasting-disease.blogspot.com/2021/11/prion-protein-polymorphisms-in-michigan.html

WEDNESDAY, OCTOBER 27, 2021

Michigan shifts approach to monitor spread of deadly deer disease deer CWD TSE Prion 220 cases confirmed in WILD to date, captive?

https://chronic-wasting-disease.blogspot.com/2021/10/michigan-shifts-approach-to-monitor.html

FRIDAY, OCTOBER 22, 2021

Michigan CWD TSE Prion TOTAL WILD CERVID 220 POSITIVE TO DATE, CAPTIVE CWD TOTAL

https://chronic-wasting-disease.blogspot.com/2021/10/michigan-cwd-tse-prion-total-wild.html

Michigan CWD TSE Prion TOTAL WILD CERVID 220 POSITIVE TO DATE, CAPTIVE CWD TOTAL???

PERSONAL COMMUNICATION DNR...see;

Mon, Aug 9, 2021 11:46 am

''The interactive hot map you are referencing is updated regularly, at least once a month, but even more regularly during hunting season as that is when we receive most samples and theoretical positives. All 209 animals to date are wild. No captive cervid deer are listed in our testing metrics because they fall into a different category of management as overseen by Michigan Department of Agriculture and Rural Development (MDARD). Because they are captive they are treated as domestic animals – same as cows, horses, and pigs. If you were inquiring about CWD+ private cervids you will have to reach out to them as we don’t so much focus on total individuals positive as we do positive herds and locations (which again they would be best to ask).''

''Our 2021 testing totals account for 11 positives this year, all USDA-APHIS or Disease Permit culled animals in Gratiot (3) and Jackson (8) counties. I am digging into when the interactive map was last updated now, but at a worst that would but our total positive at 220 over Michigan’s history of testing if it hadn’t been recently updated (which is possible as USDA-APHIS shooting just recently started up again in July after a multi-month break).''

YOU can see CWD here;

https://www.michigan.gov/emergingdiseases/0,4579,7-186-76711_78204-357110--,00.html

Where has chronic wasting disease (CWD) been found in Michigan?

Since the initial finding of CWD on May 20, 2015, free-ranging deer in Clinton, Dickinson, Eaton, Gratiot, Ingham, Ionia, Jackson, Kent and Montcalm counties have been positively confirmed with CWD. Please visit Michigan.gov/CWD for more information on CWD and the latest news and testing updates. See pages 56-57 and 61-62 for important regulations pertaining to CWD.

https://www.michigan.gov/documents/dnr/hunting_and_trapping_digest_461177_7.pdf

Assessing drivers of spread and transmission of Chronic Wasting Disease in Michigan deer

Primary Contact: Dr. Dwayne Etter, DNR Wildlife Division, Lansing, Michigan

Email: etterd@michigan.gov

Phone: (517) 284-4720

DNR Financial Support: $120,149 in FY19, $502,737 total.

Study Area: South-central Lower Peninsula

Time Frame: 10/1/2017-9/30/2022

Abstract:

The goal of this research is to improve the cost-efficiency of detecting CWD when it is still rare and removing animals from the landscape to control the spread of disease, by reducing contact among deer and potentially eliminating infectious animals. We intend to take a multi-pronged approach to accomplish this goal and the work described here will complement another study that seeks to develop new methods for detecting and removing diseased animals. The effort described here is designed to accumulate a dataset on movement behavior of deer that is of high temporal and spatial resolution to address questions about dispersal rates, directions and distances; evaluate hypotheses about environmental factors that are likely influences on dispersal behavior; parameterize risk maps of first-order contact for Michigan in concert with data and prior research in New York State; and create models of the interaction of landscape contexts (e.g., suburban, rural) and habitat characteristics that can be used to direct hunters and biologists to increase the efficiency of surveillance and removal actions. Our objectives address the strategic plans set forth by the Michigan DNR to “1.3.1: Develop and implement strategies to prevent and control diseases before they occur; 1.3.2: Respond to wildlife disease outbreaks; 1.3.4: Conduct research and monitoring to provide information to make management recommendations regarding wildlife disease; 1.3.5: Raise awareness regarding current and emerging wildlife health issues; and 1.3.6: Work with State and Federal agencies, and stakeholders to address wildlife health issues.”

12 IC4117 (Rev. 02/02/2021)

control of an emerging occurrence of CWD through better understanding of behavior and population dynamics of deer inhabiting areas of known infection.

Management of Chronic Wasting Disease in Michigan

Primary Contact: Dr. Kelly Straka, DNR Wildlife Division, Lansing, Michigan

Email: StrakaK1@michigan.gov

Phone: (517) 336-5030

DNR Financial Support: $50,000 in FY19, $250,000 total.

Study Area: Statewide.

Time Frame: 10/01/2016-09/30/2022

Quantifying Upper Peninsula deer movements and abundance: preparing for CWD management

Primary Contact: Dr. Dean Beyer Jr., DNR Wildlife Division, Marquette, Michigan

Email: beyerd@michigan.gov

Phone: (906) 228-6561

DNR Financial Support: $117,759 in FY19, $613,001 total.

Study Area: Upper Peninsula

Time Frame: 10/1/2017-9/30/2021

Abstract:

Consequently, If CWD was detected in the UP, large areas would likely be under surveillance and management that would not contain infected deer and large areas with potential for infected deer would not be within the prescribed surveillance zone, rendering the current management zone less effective. The Upper Peninsula Region (UPR), Biological and Social Sciences Section (BSSS), Wildlife Health Section (WHS), and Mississippi State University (MSU) wish to develop a program to address the need for information on deer movements. The core work would entail deploying GPS collars on deer in select wintering complexes and conditional winter range (starting along WI border) and documenting movements over three years. To complete the capture and collaring work, we would work cooperatively with interested sportspersons.

https://www.michigan.gov/documents/dnr/IC4117_SummaryofSustainableForestryResearch_652352_7.pdf

https://www.michigan.gov/dnr/0,4570,7-350-79136_79772_79773_83479---,00.html

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90541---,00.html

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90536_90552_90560---,00.html

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516---,00.html

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90536---,00.html

WEDNESDAY, SEPTEMBER 01, 2021

Michigan CWD TSE Prion 211 Cases To Date

https://chronic-wasting-disease.blogspot.com/2021/09/michigan-cwd-tse-prion-211-cases-to-date.html

Michigan CWD TSE Prion 211 Cases To Date

Deer Tested for Chronic Wasting Disease Since Detection of First Positive Free-ranging Deer (May 2015)

https://www.michigan.gov/emergingdiseases/0,4579,7-186-76711_78204-357110--,00.html

Total Deer Tested and Total Positives Cases

CWD Testing Results for Deer Harvested in 2020

CWD Testing Results for Deer Harvested in 2019

CWD Testing Results for Deer Harvested in 2018

Michigan Lower Peninsula townships where free-ranging deer have tested positive for CWD

https://www.michigan.gov/emergingdiseases/0,4579,7-186-76711_78204-357110--,00.html

Chronic Wasting Disease (CWD) Surveillance Chronic wasting disease (CWD) is a transmissible neurological disease found in deer and elk populations that produces small lesions in brains of infected animals. As a result, CWD causes weight loss and a decline in body control. It is a species-specific disease, and there have been no cases in humans or other animals.

Currently, to determine the presence of CWD, brain and lymph node samples are taken by an accredited veterinarian after an animal dies. These samples are then submitted for testing.

First, for the CWD HCP, all cervids 12 months of age and older that die for any reason must be tested for CWD.

Samples for either of these programs can be submitted to a private veterinarian, the Michigan State University Veterinary Diagnostic Lab, or an MDARD drop off location.

For more information, contact the MDARD Cervid Program.

https://www.michigan.gov/mdard/0,4610,7-125-48096_100264-530075--,00.html

Special Surveillance Area (SSA) If a free-ranging or privately-owned cervid (POC) tests positive for chronic wasting disease (CWD), then a buffer circle is created around that positive animal. This buffer circle is referred to as a Special Surveillance Area (SSA). POC facilities that fall within a SSA will have increased CWD testing requirements.

More specifically, if a free-ranging cervid is found to be positive for CWD, a 15-mile radius circle is created around the positive animal. And, if a POC is found to be positive for CWD, a 5-mile radius circle is created around the positive animal. Further, for both cases, the SSA extends to the entirety of the county of the infected animal and any county that the 15-mile or 5-mile circle touches.

For a complete list of counties that are included within a particular SSA, please review the map below.

https://www.michigan.gov/mdard/0,4610,7-125-48096_100264-530074--,00.html

Livestock Diseases:

Disease Species Number of Animals

CWD (Chronic Wasting Disease) Cervid 46

Number of herds involved in special surveillance zones around CWD positive free-ranging deer 84 84

https://www.michigan.gov/documents/mdard/2020_Animal_Industry_Division_Annual_Report_719508_7.pdf

MDARD CWD Confirmed at Farmed Deer Facilities in Mecosta and Montcalm Counties

... Rural Development (MDARD) has confirmed two cases of chronic wasting disease (CWD) at two separate farmed deer facilities, one ... 123 free-ranging deer from Montcalm County. "Since chronic wasting disease can significantly impact all Michigan deer, it is ...

https://www.michigan.gov/mdard/0,4610,7-125-1572_28248_50968-565732--,00.html

MDARD CWD Identified in Newaygo County Farmed Deer

... Agriculture and Rural and Development (MDARD) has confirmed chronic wasting disease (CWD) in three white-tailed deer from a ... rule out exposure of any other farmed deer. "Chronic wasting disease is a serious disease affecting both farmed and ...

https://www.michigan.gov/mdard/0,4610,7-125-1572_28248_50968-516936--,00.html

MDARD CWD identified in a Montcalm County farmed deer

... Rural Development (MDARD) has confirmed a case of chronic wasting disease (CWD) in a four-year-old white-tailed ... 123 free-ranging deer in Montcalm County. "As chronic wasting disease affects both farmed and free-ranging deer, MDARD ...

https://www.michigan.gov/mdard/0,4610,7-125-1572_28248_50968-554427--,00.html

Michigan CWD Confirmed at Farmed Deer Facilities in Mecosta and Montcalm Counties

CWD Confirmed at Farmed Deer Facilities in Mecosta and Montcalm Counties

For immediate release: August 11, 2021 Media contact: Jennifer Holton, 517-284-5724

LANSING, MI - The Michigan Department of Agriculture and Rural Development (MDARD) has confirmed two cases of chronic wasting disease (CWD) at two separate farmed deer facilities, one in Mecosta County and one in Montcalm County. The two infected deer, a two-year-old and a four-year-old, were discovered through routine testing as part of the state's CWD surveillance program for farmed deer.

CWD is a fatal neurological disease that affects white-tailed deer, mule deer, elk, and moose. The disease can be transmitted directly from one animal to another, as well as indirectly through the environment. While an infected deer may appear healthy for months or years, it will eventually display abnormal behavior, progressive weight loss, and physical debilitation in the latter stages of the disease.

The presence of CWD in farmed and free-ranging deer is not new to Michigan. Since 2008, and including these new cases, CWD has been detected at eight Michigan deer farms in the following counties: Kent, Mecosta (3), Montcalm (3), and Newaygo.

With free-ranging deer, CWD was first discovered in May 2015, and cases have been found across nine counties in Michigan's Upper and Lower Peninsulas. To date, while no free-ranging white-tailed deer have tested positive for CWD in Mecosta County, the disease has been detected in 123 free-ranging deer from Montcalm County.

"Since chronic wasting disease can significantly impact all Michigan deer, it is vitally important to detect the disease as early as possible," said State Veterinarian Nora Wineland, DVM. "Early detection allows MDARD and the Michigan Department of Natural Resources to work in collaboration with farmers and hunters to stem the spread and manage this serious disease."

As part of MDARD's disease response, investigations are being conducted to rule out exposure to any other farmed deer.

Currently, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend that infected animals should not be consumed as food by either humans or domestic animals.

More information about CWD can be found at Michigan.gov/CWD or Michigan.gov/MDARD-Cervid.

###

https://www.michigan.gov/mdard/0,4610,7-125--565732--,00.html

CWD identified in a Montcalm County farmed deer

For immediate release: March 12, 2021 Media contact: Jessy Sielski, 517-331-1151

LANSING, MI - The Michigan Department of Agriculture and Rural Development (MDARD) has confirmed a case of chronic wasting disease (CWD) in a four-year-old white-tailed deer from a Montcalm County deer farm. The case was found through samples that were submitted for routine testing as part of the state's CWD surveillance program for farmed deer.

CWD is a fatal neurological disease that affects white-tailed deer, mule deer, elk, and moose. CWD can be transmitted directly from one animal to another, as well as indirectly through the environment. While an infected deer may appear healthy for months or years, it will eventually display abnormal behavior, progressive weight loss, and physical debilitation in the latter stages of the disease.

The discovery of CWD in farmed and free-ranging deer is not new to the state of Michigan. Since 2008, and including this new case, CWD has been detected at six Michigan deer farms in the following counties: Kent, Mecosta (2), Montcalm (2), and Newaygo.

With free-ranging deer, CWD was first discovered in May 2015, and cases have been found across nine counties in both the Upper and Lower Peninsulas. To date, CWD has been detected in 123 free-ranging deer in Montcalm County.

"As chronic wasting disease affects both farmed and free-ranging deer, MDARD works in partnership with the Michigan Department of Natural Resources and the state's deer farmers to detect and manage this serious disease," said State Veterinarian Nora Wineland, DVM. "Due to the nature of the disease, it is imperative that farmers, hunters, DNR, and MDARD continue to work in collaboration to protect all of Michigan's deer."

As part of MDARD's disease response, an investigation will be conducted to rule out exposure of any other farmed deer.

Currently, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend infected animals not be consumed as food by either humans or domestic animals.

More information about CWD can be found at Michigan.gov/CWD or Michigan.gov/MDARD-Cervid.

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https://www.michigan.gov/mdard/0,4610,7-125-1572_28248_50968-554427--,00.html

APPENDIX A: 2020 REPORTABLE DISEASES

Livestock Diseases:

CWD (Chronic Wasting Disease) Cervid 46

https://www.michigan.gov/documents/mdard/2020_Animal_Industry_Division_Annual_Report_719508_7.pdf

CWD Identified in Newaygo County Farmed Deer

For Immediate Release: January 14, 2020 Media Contact: Jessy Sielski, 517-284-5725

LANSING, MI - The Michigan Department of Agriculture and Rural and Development (MDARD) has confirmed chronic wasting disease (CWD) in three white-tailed deer from a Newaygo County deer farm. All three deer were four-and-a-half years old. The samples were submitted for routine testing as part of the state's CWD surveillance program for farmed deer.

To date, CWD has not been detected in free-ranging deer in Newaygo County. As part of MDARD's disease response, an investigation will be conducted to rule out exposure of any other farmed deer.

"Chronic wasting disease is a serious disease affecting both farmed and free-ranging deer," said State Veterinarian Nora Wineland, DVM. "MDARD and the Michigan Department of Natural Resources work together, in partnership with the state's deer farmers, to ensure the protection of all of Michigan's deer."

Since 2008, CWD has been detected in four additional privately-owned cervid facilities from Kent, Mecosta, and Montcalm Counties. The deer farm in Newaygo County is the fifth Michigan farm in which CWD has been detected.

CWD is a fatal neurological disease that affects white-tailed deer, mule deer, elk, and moose. CWD can be transmitted directly from one animal to another, as well as indirectly through the environment. Infected animals may display abnormal behavior, progressive weight loss and physical debilitation. To date, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend that infected animals not be consumed as food by either humans or domestic animals.

More information about CWD can be found at Michigan.gov/CWD.

https://www.michigan.gov/mdard/0,4610,7-125-1572_28248_50968-516936--,00.html

https://www.michigan.gov/documents/mdard/May_15_2019_Commission_Meeting_Materials_655387_7.pdf

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516---,00.html

Total Deer Tested and Total Positives Cases

CWD Testing Results for Deer Harvested in 2020

CWD Testing Results for Deer Harvested in 2019

CWD Testing Results for Deer Harvested in 2018

Michigan Lower Peninsula townships where free-ranging deer have tested positive for CWD

https://www.michigan.gov/emergingdiseases/0,4579,7-186-76711_78204-357110--,00.html

Greenville man charged with violating CWD deer requirements

By Elisabeth Waldon | on February 03, 2021

Eric Snyder

EUREKA TOWNSHIP — A Greenville man is facing half a dozen charges related to his alleged improper handling of deer with chronic wasting disease (CWD).

Eric David Snyder, 51, is charged with three counts of animal industry acts (privately owned cervidae) two counts of animal industry acts (felony violation) and one count of animals burial.

According to Montcalm County Prosecutor Andrea Krause, the alleged crimes occurred between March 2019 and February 2020. The Department of Natural Resources investigated, however, Snyder wasn’t charged until December 2020 and he wasn’t arraigned until Jan. 21. Krause said the coronavirus pandemic likely played a role in the delay.

According to Krause, Snyder owned Fieldview Whitetails, a deer farm/ranch in Eureka Township.

“He had a deer test positive for CWD,” Krause said. “(Snyder) dumped the deer outside the farm in violation of the law. He also violated a quarantine of the other deer he had on his farm.”

A deer with CWD in Montcalm County was discovered in October 2017 and as a precaution in January 2018, all privately owned deer facilities were put into mandatory quarantine if they were within 15 miles of a deer that tested positive, according to Krause. In March 2019, a deer at Snyder’s farm was discovered to have CWD, and a follow-up investigation later that month discovered the alleged violations at the farm, according to Krause.

Snyder is being represented by attorney Jeff Crampton. If convicted, Snyder faces to from 90 days to five years prison and/or fines and costs.

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https://thedailynews.cc/articles/greenville-man-charged-with-violating-cwd-deer-requirements/

MONDAY, JUNE 28, 2021

Michigan Total CWD Positive/Suspect Positive Deer 209 Cases To Date

https://chronic-wasting-disease.blogspot.com/2021/06/michigan-total-cwd-positivesuspect.html

FRIDAY, NOVEMBER 27, 2020

Michigan, to date, CWD TSE Prion has been detected in 197 cervid

https://chronic-wasting-disease.blogspot.com/2020/11/michigan-to-date-cwd-tse-prion-has-been.html

SUNDAY, OCTOBER 11, 2020

Michigan Chronic Wasting Disease CWD TSE Prion increases to 191 positive to date

https://chronic-wasting-disease.blogspot.com/2020/10/michigan-chronic-wasting-disease-cwd.html

TUESDAY, SEPTEMBER 22, 2020

Michigan CWD TSE Prion 189 Positive To Date UPDATE September 2020

https://chronic-wasting-disease.blogspot.com/2020/09/michigan-cwd-tse-prion-189-positive-to.html

WEDNESDAY, MARCH 25, 2020

Michigan CWD TSE Prion Total Suspect Positive Deer Moves Up To 188 with total deer tested 80,687 to date

https://www.michigan.gov/emergingdiseases/0,4579,7-186-76711_78204-357110--,00.html

https://chronic-wasting-disease.blogspot.com/2020/03/michigan-cwd-tse-prion-total-suspect.html

THURSDAY, JANUARY 30, 2020

Michigan CWD TSE Prion Total Suspect Positive Deer Jumps To 181 to date

https://chronic-wasting-disease.blogspot.com/2020/01/michigan-cwd-tse-prion-total-suspect.html

MONDAY, JANUARY 27, 2020

Michigan CWD TSE Prion MDARD 3 positive white-tailed deer from a Newaygo County deer farm depopulation and quarantine efforts update?

https://chronic-wasting-disease.blogspot.com/2020/01/michigan-cwd-tse-prion-mdard-3-positive.html

TUESDAY, JANUARY 14, 2020

Michigan MDARD has confirmed chronic wasting disease (CWD) in 3 white-tailed deer from a Newaygo County deer farm

https://chronic-wasting-disease.blogspot.com/2020/01/michigan-mdard-has-confirmed-chronic.html

TUESDAY, JANUARY 07, 2020

Michigan Total CWD TSE Prion Positive Suspect-Positive Deer Jump To 174 confirmed to date

https://chronic-wasting-disease.blogspot.com/2020/01/michigan-total-cwd-tse-prion-positive.html

THURSDAY, DECEMBER 12, 2019

Michigan Total CWD TSE Prion Positive Suspect-Positive Deer Jump To 162 confirmed to date

https://chronic-wasting-disease.blogspot.com/2019/12/michigan-total-cwd-tse-prion-positive.html

FRIDAY, NOVEMBER 22, 2019

Michigan Total CWD TSE Prion Positive/Suspect-Positive Deer 140 To Date

https://chronic-wasting-disease.blogspot.com/2019/11/michigan-total-cwd-tse-prion.html

WEDNESDAY, NOVEMBER 06, 2019

Michigan Total CWD TSE Prion Positive, Suspect Positive, Deer 136 To Date

https://chronic-wasting-disease.blogspot.com/2019/11/michigan-total-cwd-tse-prion-positive.html

2019 CWD Testing Goals and Results as of October 18, 2019

https://www.michigan.gov/dnr/0,4570,7-350-79136_79608_90516_90536-501527--,00.html

THURSDAY, OCTOBER 24, 2019

Michigan DNR reports CWD-positive deer in Hamilton Township, Gratiot County

https://chronic-wasting-disease.blogspot.com/2019/10/michigan-dnr-reports-cwd-positive-deer.html

THURSDAY, OCTOBER 24, 2019

Michigan DNR reports CWD-positive deer in Hamilton Township, Gratiot County

http://chronic-wasting-disease.blogspot.com/2019/10/michigan-dnr-reports-cwd-positive-deer.html

SATURDAY, OCTOBER 05, 2019

Michigan MSU SCIENTISTS ARE TESTING A FASTER WAY TO DETECT CHRONIC WASTING DISEASE

https://chronic-wasting-disease.blogspot.com/2019/10/michigan-msu-scientists-are-testing.html

SUNDAY, SEPTEMBER 22, 2019

Michigan TWO MORE CWD TSE PRION POSITIVES Total Now At 124 Positive

https://chronic-wasting-disease.blogspot.com/2019/09/michigan-two-more-cwd-tse-prion.html

TUESDAY, SEPTEMBER 17, 2019

Michigan House Bill 4687 State Legislators Turn To Draft Dodger Ted Nugent To Make Scientific Decisions over DNR on CWD TSE Prion

https://chronic-wasting-disease.blogspot.com/2019/09/michigan-house-bill-4687-state.html

SATURDAY, AUGUST 24, 2019

Michigan Chronic Wasting Disease CWD TSE Prion Two More Cases Total 122 To Date

https://chronic-wasting-disease.blogspot.com/2019/08/michigan-chronic-wasting-disease-cwd.html

THURSDAY, MAY 23, 2019

Michigan Osceola County deer farm/ranch owner arraigned on several violations

https://chronic-wasting-disease.blogspot.com/2019/05/michigan-osceola-county-deer-farmranch.html

THURSDAY, MAY 09, 2019

Michigan CWD TSE Prion increases to 120 Cases to Date

https://chronic-wasting-disease.blogspot.com/2019/05/michigan-cwd-tse-prion-increases-to-120.html

THURSDAY, MARCH 28, 2019

Michigan CWD Identified in a Montcalm County Farmed Deer

https://chronic-wasting-disease.blogspot.com/2019/03/michigan-cwd-identified-in-montcalm.html

March 30, 2018

Contact: Lt. David Shaw, 616-218-3762

Mecosta County man sentenced following DNR investigation

Game ranch owner falsified information related to chronic wasting disease testing

A Mecosta County game ranch owner has been sentenced on charges resulting from an investigation by the Michigan Department of Natural Resources Law Enforcement Division, in cooperation with the Michigan Department of Agriculture and Rural Development.

Lester Jay Gemmen, 64, of Morley was charged with providing false information regarding the origin of two deer heads that were submitted for disease testing, and for failing to properly maintain fencing at the Super G Ranch. The ranch is a privately owned cervid (POC) facility, a designation that includes game ranches and hunting ranches.

He was sentenced by the 77th District Court to 60 days in jail for each count, ordered to pay $775 in fines and costs and must perform 80 hours of community service.

The investigation began in 2017 after two of the six deer heads submitted by Gemmen tested positive for chronic wasting disease (CWD).

“I commend the detectives from our Special Investigations Unit and our field conservation officers for their thorough, professional approach to this investigation,” said 1st Lt. David Shaw, supervisor of the Special Investigations Unit of the DNR Law Enforcement Division.

The facility’s remaining deer were depopulated and tested, but no further evidence of CWD was found. The facility remains under quarantine, currently preventing ownership of farmed cervids.

The Privately Owned Cervid Program is jointly managed by the DNR and MDARD. There is mandatory CWD testing in all registered herds in Michigan, under the oversight of MDARD. The DNR oversees POC registration and performs inspections of POC facilities. Proper maintenance of POC facilities is critical to protecting Michigan’s free-ranging and privately owned cervid herds.

CWD is a fatal central nervous system disease that affects white-tailed deer, mule deer, elk and moose. It attacks the brain of infected animals, creating small lesions in the brain, which result in death. It is transmitted through direct animal-to-animal contact or by contact with saliva, urine, feces, blood, carcass parts of an infected animal or infected soil. To date, there have been no reported cases of CWD infection in humans. However, as a precaution, the U.S. Centers for Disease Control and Prevention and the World Health Organization recommend that infected animals not be consumed as food by humans or domestic animals.

Since May 2015, CWD-positive deer have been found in Michigan. As of mid-March 2018, 57 free-ranging deer have tested positive for the disease. CWD has not been found in the Upper Peninsula, though it has been discovered in Wisconsin, approximately 40 miles from the western Upper Peninsula border.

The DNR is working with stakeholders to address the status of CWD in Michigan. In the coming weeks, the DNR and the Michigan Natural Resources Commission will host a series of public engagement meetings across the state on CWD. The sessions will provide hunters, business owners and residents with opportunities to share their ideas and observations.

In addition, the DNR, NRC and MDARD are evaluating recommendations from the CWD Working Group, which was created after last year’s CWD Symposium. The symposium brought national and international experts to Michigan to discuss CWD. During the coming months, the DNR, NRC and MDARD will work with stakeholders to develop new CWD regulation recommendations.

Visit www.michigan.gov/cwd for more information about the disease, preventive measures and the public meeting schedule.

https://content.govdelivery.com/accounts/MIDNR/bulletins/1e60b39

SATURDAY, MARCH 03, 2018

Michigan-Sportsman.com Bans Terry S. Singeltary Sr. For Speaking about Chronic Wasting Disease CWD TSE Prion

https://www.michigan-sportsman.com/threads/chronic-wasting-disease-cwd-tse-prion-roundup-february-18-2018.609396/

https://www.michigan-sportsman.com/threads/chronic-wasting-disease-cwd-tse-prion-roundup-february-18-2018.609396/page-2

https://www.usgs.gov/media/images/distribution-chronic-wasting-disease-north-america-0

Voluntary Chronic Wasting Disease Herd Certification Program Annual Update, FY2020

Last Modified: Feb 9, 2021

U.S. Department of Agriculture

Animal and Plant Health Inspection Service (APHIS) Veterinary Services

Annual Update from the Cervid Health Team

Voluntary Chronic Wasting Disease Herd Certification Program (HCP)

The APHIS National CWD Herd Certification Program (HCP) was implemented in 2014. It is a voluntary Federal-State-industry cooperative program administered by APHIS and implemented by participating States. The program provides uniform national herd certification standards that minimize the risk of spreading CWD in farmed cervid populations. Participating States and herd owners must comply with requirements for animal identification, fencing, recordkeeping, inspections/inventories, as well as animal mortality testing and response to any CWD-exposed, suspect, and positive herds. APHIS monitors the Approved State HCPs to ensure consistency with Federal standards through annual reporting by the States.

With each year of successful surveillance, herds participating in the HCP will advance in status until reaching five years with no evidence of CWD, at which time herds are certified as being low risk for CWD. Only farmed cervids from enrolled herds certified as low risk for CWD may move interstate. FY 2020 marks the eighth year that Approved States have submitted their CWD HCP annual reports to APHIS.

The current Cervid Health Program staff officers are as follows: Dr. Mark Lyons, Dr. Jennifer Siembieda, and Dr. Tracy Nichols

Voluntary Herd Certification Participation Summary

Currently, 28 States participate in the voluntary CWD Herd Certification Program encompassing 2,145 enrolled herds, of which, 1,723 had the certified status in the program.

1,616 enrolled deer herds, of which, 1,297 were certified

371 enrolled elk herds, of which, 328 were certified

147 enrolled mixed species herds, of which, 98 were certified

CWD in Farmed Cervids Summary of CW Detections

There were 22 newly identified CWD positive herds in FY20

13 of these herds were not participants in the Federal HCP

2 herds were considered enrolled in the HCP

7 herds were certified in the HCP

Half of the herds were located within 20 miles of identified CWD in the wild, half were not CWD Herds by State

Pennsylvania: Eight new CWD positive herds

Breeding herd of 33 WTD, HCP certified, depopulated with Federal indemnity

Breeding herd of 6 WTD, not in HCP, depopulated with Federal indemnity

Breeding herd of 15 WTD, not in HCP, depopulated by owner\

Hunt preserve of 58 WTD, not in HCP, populated and under quarantine

Breeding herd of 75 WTD, not in HCP, populated and under quarantine

Breeding herd of WTD, not in HCP, populated and under quarantine

Breeding herd of 90 WTD, not in HCP, populated and under quarantine

Breeding herd of 4 WTD, not in HCP, populated and under quarantine

Iowa: Two new CWD positive herds

Breeding herd of 23 WTD, HCP certified, depopulated with Federal indemnity

Breeding herd of 13 WTD, HCP certified, depopulated with Federal indemnity

Minnesota: Two new CWD positive herds

Breeding herd of 3 WTD, enrolled in HCP, not certified, depopulated by owner

Breeding herd of 6 WTD, enrolled in HCP, not certified, depopulated with Federal indemnity

Colorado: Two new CWD positive herds

Breeding herd/hunt preserve of 9 elk, HCP certified, depopulated by owner

Breeding herd of 8 elk, HCP certified, populated and under quarantine

Utah: Two new CWD positive herds

Breeding herd of 465 elk, not in HCP, partial depopulation with Federal indemnity- removed purchased animals, populated-quarantine

Breeding herd of 103 elk, not in HCP, partial depopulation with Federal indemnity- removed purchased animals, populated-quarantine

Michigan: One new CWD positive herd

Hunt preserve of >600 WTD, not in HCP, populated and under quarantine

Montana: One new CWD positive herd

Breeding herd of 3 elk, not in HCP, populated and under quarantine

Texas: one new CWD positive herd

Breeding herd of 59 WTD, not in HCP, depopulated with Federal indemnity

Kansas: One new CWD positive herd

Breeding herd of 20 elk, HCP certified, depopulated with Federal indemnity

Ohio: Eight new CWD positive herd

Breeding herd of 138 WTD, HCP certified, depopulated with Federal indemnity

Research

Whole genome study investigating the association of genetics with CWD susceptibility has been published.

Blinded validation of the genetic predicative model is almost complete

A standardized protocol has been developed, in partnership with ARS, USGS, University of WI, and NIH for tissue sample testing using RT-QuIC

A study is starting shortly to determine the sensitivity and specify of RT-QuIC utilizing the standardized protocol

snip...

Voluntary Chronic Wasting Disease Herd Certification Program Annual Update, FY2020

https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/voluntary-cwd-hcp-annual-update-fy2020

Cervids: CWD Voluntary Herd Certiﬁcation Program

Last Modified: Jun 29, 2021

https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-disease-information/cervid/cervids-cwd/cervids-voluntary-hcp

CWD status of captive herds

https://www.aphis.usda.gov/animal_health/animal_diseases/cwd/downloads/status-of-captive-herds.pdf

SUNDAY, OCTOBER 24, 2021

Voluntary Chronic Wasting Disease Herd Certification Program Annual Update, FY2020

https://chronic-wasting-disease.blogspot.com/2021/10/voluntary-chronic-wasting-disease-herd.html

USDA Transmissible Spongiform Encephalopathy TSE Prion Action Plan National Program 103 Animal Health 2022-2027

USDA TSE PRION Action Plan National Program 103 Animal Health 2022-2027

Action Plan National Program 103 Animal Health 2022-2027

SNIP...

Component 6: Transmissible Spongiform Encephalopathies (TSEs)..................................... 37

Problem Statement 6A: Determine pathobiology of prion strains...................................... 39

Problem Statement 6B: Reveal genetics of prion disease susceptibility............................. 40

Problem Statement 6C: Diagnose, detect, and prevent prion diseases................................ 41

Appendix 1: Meetings with livestock and government stakeholders that informed the development of this action plan................................................................................................ 44

Appendix 2: Scientific and government stakeholder input that informed this action plan ..... 45

Appendix 3: Heatmap of responses across all survey respondents to the 10 most important diseases currently affecting or that have the potential of affecting animal agriculture in the United States (n=413):.............................................................................................................. 46

SNIP...

Component 6: Transmissible Spongiform Encephalopathies (TSEs)

Transmissible spongiform encephalopathies (TSEs) include several fatal diseases of people and animals involving degeneration of the nervous system and brain function. TSEs are caused by agents known as prions, or what appear to be primarily infectious proteins that cause normal protein (cellular-prion protein PrPc ) molecules to convert into an abnormally structured form (disease-prion protein PrPsc) that can include inducement of the formation of proteinaceous deposits and plaques in the brain. TSEs include Creutzfeldt-Jakob disease (CJD), the primary human prion disease; Scrapie of sheep and goats; Chronic Wasting Disease (CWD) of deer, elk, and moose; and Bovine Spongiform Encephalopathy (BSE), also called “mad cow,” which is the cause of variant CJD (vCJD) in people and the only TSE known to have crossed the species barrier from animals to people.

Our understanding of TSEs continues to evolve with ongoing research efforts. TSEs are progressive but long developing diseases. In humans, for example, incubation periods from

the time of contact with an infectious prion may be decades long. Consequently, completion of research plans in natural hosts may require several years. Improvements have been made with the development of experimental rodent models to shorten the time required to obtain experimental results, but the relevance of any findings in mouse models remains uncertain unless confirmed and validated in natural hosts. In 2004, the Institute of Medicine of the National Academies published a report entitled: Advancing Prion Science, Guidance for the National Prion Research Program. Several federal agencies have directed resources to implement recommendations in the report, including HHS-NIH, USDA-ARS, HHS-FDA, HHS-CDC, DoD, and EPA. Although significant scientific advances have been made, the research conducted to date has yet to deliver many of the concrete solutions needed to safeguard people and animals from these devastating diseases. A critical concern is the potential for environmental, genetic, or iatrogenic events to lead to new variant TSEs that are infectious and zoonotic.

The White House Office of Science and Technology Policy (OSTP) Interagency Working Group (IWG) on Prion Science identified the following research priorities to maximize the impact of the National Prion Research Program:

• Identification of the nature and origin of prion agents

• Studies on the pathobiology of prion strains

• Research on the determinants of transmissibility and epidemiology

• Development of diagnostics, detection, and surveillance

These interrelated priorities represent areas with critical gaps in our knowledge base. They were selected with the aim of establishing strategic collaborations that will produce benefits by aligning core competencies across Federal agencies. Especially notable are the potential benefits to be derived from collaboration between animal health and human-biomedical research.

All sectors that completed the 2020 ARS Animal Health Stakeholder Survey (government, academia, industry, and livestock and poultry producers) identified research on TSEs a national priority. Importantly, stakeholders identified the following TSEs as one of the 10 most important diseases that have the potential of significantly affecting animal agriculture in the United States: Chronic Wasting Disease (29%), Bovine Spongiform Encephalopathy (18%), and Scrapie (11%).

Producers that completed the 2020 ARS Animal Health Stakeholder Survey (beef, sheep, goats, and wildlife) also identified TSEs as one of the top five diseases currently affecting their commodity. The following TSEs were ranked by producers as one of the top five diseases, as follows:

Chronic Wasting Disease: Wildlife, including captive cervids (80%), sheep (15%), and beef (4.3%).

Bovine Spongiform Encephalopathy: Beef (8.7%).

Scrapie: Sheep (56%), goats (17%), beef (4.3%).

Although recognized as important, ARS does not currently have resources to implement research for the following priority:

• Identification of the nature and origin of prion agents.

Problem Statement 6A: Determine pathobiology of prion strains

Important gaps remain in our basic understanding of the pathobiology of animal prion diseases. One critical need is understanding the tissue tropism and dissemination of prions and resolving the variations seen in different animal species. Proving especially problematic is that the normal prion protein is widely expressed, particularly on neurons in the brain, and is found on cell surfaces but its function is unclear. Another enigma of TSEs is that different strains are found within the same animal species. Importantly, there is evidence that atypical strains have emerged and there is a need to investigate the transmissibility of atypical Scrapie strains, such as the Nor98-like Scrapie.

Research Focus:

It is widely assumed that the oral route of infection is important in the pathogenesis of naturally occurring TSEs of livestock and cervids; however, basic research is needed to understand the mechanisms of transmission of TSE agents from the initial site of entry to the central nervous system. A notable feature of prion diseases is a lack of detectable immune responses and inflammation during the course of a prion infection, even though immune system cells may carry prions to target tissues. To date, research in animals suggests that prion accumulation may be largely influenced by the host species affected rather than the TSE involved. An investment in comparative pathology, which has not received much experimental attention, is needed to advance research programs in epidemiology and diagnostic discovery.

Anticipated Products:

• Scientific information on the mechanisms responsible for the development of multiple TSE strains within a host species.

• Scientific information on the manner in which prions enter the nervous system from peripheral sites of exposure such as a host’s gastrointestinal tract, nasal mucosa, skin, and eyes.

• Scientific information on the mechanisms by which prion spread within the nervous system.

• Scientific information on the mechanisms that control prion disease incubation times.

• Elucidate the mechanisms of prion neuropathogenesis.

• Determine prion distribution in goats infected with Scrapie.

• Scientific information on prion distribution in sheep infected with atypical Scrapie.

Potential Benefits:

• Understanding the pathobiology of prion disease and tissue distribution in susceptible animal species is paramount to inform the development of detection methods and ability to develop countermeasures to protect against animal prion diseases.

Problem Statement 6B: Reveal genetics of prion disease susceptibility

Prion diseases have stimulated intense scientific scrutiny since it was first proposed that the infectious agent was devoid of nucleic acid. Despite this finding, host genetics has played a key role in understanding the pathobiology and clinical aspects of prion diseases through the effects of a series of polymorphisms and mutations in the prion protein gene. The advent of vCJD confirmed a powerful human genetic susceptibility factor, as all patients with clinical disease have an identical genotype at the polymorphic codon 129 of the prion gene. The alternative variant at codon 129 is not protective, however, and abnormal prions have been found in lymphoid tissues of individuals of other prion genotypes after exposure to transfused blood products from patients who later succumbed to the disease. Familial forms of prion diseases are also known to be inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person inherits the altered gene from one affected parent. In some people, familial forms of prion disease are caused by a new mutation in the prion gene. Although such people most likely do not have an affected parent, they can pass the genetic change to their children. Familial Creutzfeldt-Jakob disease (fCJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia (FFI) represent the core phenotypes of genetic prion disease.

Genetic studies in animals have uncovered similar polymorphisms and mutations in the prion protein gene. Genetic information has led to the discovery of genotypes with relative susceptibility and resistance to Scrapie in sheep. Current Scrapie control programs in the United States and Europe are based on the elimination of susceptible genotypes from the breeding pool. However, a significant portion of Scrapie resistance in sheep is not explained by the currently known resistance allele coding R, at codon 171, and codon A at codon 136. Less is known in cervids and CWD. In addition, recent evidence indicates that some forms of BSE may be genetic in nature. The 2006 U.S. H.-type atypical BSE cow had a polymorphism at codon 211 of the bovine prion gene, resulting in a glutamic acid to lysine substitution (E211K). This substitution is analogous to a human polymorphism associated with the most prevalent form of heritable TSE in humans, and it is considered to have caused BSE in 2006 in a U.S. case that was determined to be atypical BSE.

Research Focus:

The functional genomics of disease resistance are not completely understood, and recent research suggests genetic variations may lead to different clinical outcomes. There is a need to look more broadly at the genome of livestock species to identify markers associated with resistance to Scrapie in sheep and goats and CWD in cervids.

In the case of Scrapie, the sheep genome may help identify other alleles that may explain why some QR and RR sheep genotype are susceptible, allowing these sheep to be classified as susceptible and removed from the farm. This will make genotype testing a more effective control tool. This research area is aimed at utilizing powerful computational biology and bioinformatic approaches, along with traditional animal breeding experiments, to steadily improve our understanding of mechanisms of genetic disease resistance.

Our understanding of Scrapie genetic resistance in goats is not as advanced as sheep Scrapie, and there is a need to identify markers for genetic resistance in goats. This will enable the use

of markers identified to develop resistant lines of high production meat and milk goats in cooperation with industry. The USDA eradication program is increasing its focus on goats and it is critically important to provide other options to goat producers besides whole herd depopulation, with the hope that premises contamination does not result in reinfection. Scrapie eradication in the United States will not be achieved unless it is eradicated from sheep and goats.

Anticipated Products:

• Identification of genetic variations associated with disease susceptibility.

• Scientific information on the correlation between host genotypes and the phenotypes of prion agents.

• Identification of genetic factors controlling susceptibility of goats to Scrapie.

• Scientific information to evaluate the effectiveness of disease resistance breeding programs in sheep.

• Scientific information to evaluate sheep ARR/ARR genotype for resistance to different TSE strains.

• Determine whole genome associations with TSE susceptibility or resistance in sheep, goats, and cervids.

• Determine the effects of the PRNP genotype on current diagnostic test assay accuracy in sheep and goats with Scrapie.

Potential Benefits:

• The identification of genetic markers associated with disease susceptibility and resistance.

• Ability to develop prion disease control programs by selecting farm animals that are resistant to prion diseases.

• Ability to enhance surveillance programs for animals known to be genetically susceptible to prion diseases.

Problem Statement 6C: Diagnose, detect, and prevent prion diseases

Important gaps remain in our arsenal of diagnostic tools for early detection and countermeasures to prevent disease outbreaks, transmission, and spread. Current diagnostic tests were validated for use only on post-mortem samples; simple, sensitive, cost-effective ante mortem tests have yet to be developed. Because there is no detectable immune response or inflammation during the course of TSE infection, direct tests are needed to confirm a diagnosis. At present, only highly infected tissues, such as brain material or lymph tissue, are suitable for providing accurate diagnosis.

There is also a need to determine what level of environmental contamination can lead to infections in animals, and then develop a test for determining if this level of contamination exists on farm premises.

Significant gaps also remain for inactivating TSEs in farm settings. Currently the methods available for prion inactivation are not very effective in soil and other organic material. This is problematic as most contaminated bedding is either buried, left as is, or tilled in the soil relying on exclusion or dilution. Research studies have shown that prions last a very long

time when bound to soils or water and may be taken up by plants. Development of a costeffective method of prion inactivation to non-transmissible levels is needed.

Research Focus:

Diagnostic approaches currently in use include techniques such as immunohistochemistry (IHC), Western blot, and enzyme-linked immunosorbent assays (ELISA). IHC is one of the original tests developed and is considered the gold standard, but it is more labor intensive and time consuming than the other two, whereas the Western blot and particularly ELISA tests are more efficient for the initial screening of large numbers of samples. Another method is the Conformation-Dependent Immunoassay (CDI), currently a research technique that claims to discriminate between normal prion and the abnormal prion on the basis of its shape, but this has yet to be validated as a diagnostic test in animals. New technologies and methods have been described using protein misfolding cyclic amplification techniques (PMCA), similar in concept to gene/DNA amplification, which effectively increases the concentration of prions in normal or pathological conformations. There is a critical need to improve diagnostics methods for surveillance, including the discovery of an ante mortem test for early detection and implementation of intervention strategies. There is also a critical need to develop tools for inactivating TSEs in farm settings, especially the inactivation of TSEs present in organic material.

Anticipated Products:

• TSE diagnostic test capable of detecting low levels of abnormal prions (i.e., key step to enable the development of an ante mortem test that can identify disease during the early stages of incubation).

• Improved live animal and post mortem tests for Scrapie.

• Develop a sensitive, high-throughput assay suitable for use in veterinary diagnostic laboratories for detection of PrP-Sc in sheep with classical scrapie.

• Develop a live animal test for the early detection of CWD in white tail deer.

• Validation of existing biopsy-based TSE tests in goats, deer, and elk.

• Standardize sampling and assay protocols for screening environments for CWD and Scrapie prions.

• Rapid biochemical methods for strain typing.

• Determine the suitability of a sensitive, high-throughput assay for detection of PrP-Sc (Nor98) in brain, peripheral tissues, and placentas from Sheep with Nor98.

• Validated murine models for strain typing.

• Improved diagnostics for TSEs in bodily fluids, including blood and other readily available samples in host species.

• Technologies to distinguish infectious prions from normal cellular prion proteins.

• A sensitive, high-throughput assay suitable for use in veterinary diagnostic laboratories for detection of PrP-Sc in sheep with classical scrapie.

• Effective chemicals with anti-prion properties that can safely be used in farm environments.

Potential Benefits:

• Effective surveillance programs based on early detection of animal prion diseases.

• Deployment of animal prion disease prevention measures.

Component 6 Resources:

The following ARS locations have research projects addressing the problem statements

identified under Component 7:

• Albany, California

• Ames, Iowa

• Pullman, Washington

SNIP...

https://www.ars.usda.gov/ARSUserFiles/np103/Action%20Plan%20-%20Animal%20Health%202022-2027_final.pdf

''This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. 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.''

SATURDAY, OCTOBER 8, 2022

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

https://bovineprp.blogspot.com/2022/10/cattle-with-ek211-prnp-polymorphism-are.html

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 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.

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. PrPScwas detected in brain tissue by enzyme immunoassay with an average optical density of 3.6/4.0 for positive brains. PrPScalso was detected in brain tissue by western blot and immunohistochemistry. No PrPScwas 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 PrPScusing 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 CJDElkPrPin transgenic mice expressing human PrP (Tg40h). We passaged CJDElkPrPtwo more times in the Tg12 mice. We found that such second and third passage CJDElkPrPprions 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 CJDElkPrPshare 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 PrPCamino 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. PrPCWDbrain 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 PrPCprimary 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 PrPCinto PrPScin vitro by serial protein misfolding cyclic amplification (sPMCA) but also to determine whether the sPMCA-induced CWD-derived human PrPScis 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 PrPScand neuropathological changes of inoculated animals.

Results: We report here the generation of the first CWD-derived infectious human PrPScusing elk CWD PrPScto initiate conversion of human PrPCfrom normal human brain homogenates with PMCA in vitro. Western blotting with a human PrP selective antibody confirmed that the PMCA-generated protease-resistant PrPScwas derived from the human brain PrPCsubstrate. Two lines of humanized transgenic mice expressing human PrPCwith 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 PrPScpatterns and neuropathological changes in the brain.

Conclusions: Our study, using PMCA and animal bioassays, provides the first evidence that CWD PrPSchas the potential to overcome the species barrier and directly convert human PrPCinto infectious PrPScthat 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

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 PrPCtransgenic 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)

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

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.

Funded by: USDA Grant number: AP20VSSPRS00C143

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

Protein misfolding cyclic amplification (PMCA) as an ultra-sensitive technique for the screening of CWD prions in different sample types

Francisca Bravo‐Risia,b, Paulina Sotoa,b, Rebeca Benaventea, Hunter Reedc, Mitch Lockwoodc, Tracy Nicholsd, and Rodrigo Moralesa,b aDepartment of Neurology, The University of Texas Health Science Center at Houston, Houston, TX, USA; bCentro Integrativo de Biologia y Quimica Aplicada (CIBQA), Universidad Bernardo O’Higgins, Santiago, Chile; cTexas Park and Wildlife Department, Texas, USA; dVeterinary Services Cervid Health Program, United States Department of Agriculture, Animal and Plant Health Inspection Service, Fort Collins, Colorado, USA

Chronic wasting disease (CWD) is a prion disease that affects farmed and free-ranging cervids. The infectious agent in CWD is a misfolded form of the prion protein (PrPSc) that promotes conformational changes in the host’s cellular prion protein (PrPC). Currently, definitive CWD status is confirmed in the brain and lymphoid tissues by immunohistochemistry. The limitation of this technique is its poor sensitivity. Protein misfolding cyclic amplification (PMCA) and real-time quaking-induced conversion (RT- QuIC) are ultra-sensitive techniques that overcome these issues. PMCA mimics the self- propagation of infectious prions in vitro through multiple incubation/sonication cycles, increasing the number of prion particles present in a given sample. The detection of proteinase K (PK) -resistant PrPScby PMCA has been performed in experimental and natural samples that might harbor subclinical levels of prions. These samples include several tissues, bodily fluids, excreta, and different manmade and natural materials, including mineral licks, soils, and plants. Aims: In this study, we highlight recent advances and contributions that our group has performed in the detection of CWD prions from samples collected in farmed and free-ranging cervids, as well as other specimens involving the environment that contains CWD-infected deer. Material and Methods: A set of diverse samples analyzed in this study were collected by USDA and TPWD personnel in breeding and taxidermy facilities, and deer breeding facilities. These included animal and environmental samples. Additional samples from free-ranging animals were provided by hunters. Results: The diverse range of samples successfully detected for CWD prion infection in this study include blood, semen, feces, obex, retropharyngeal lymph node, fetuses (neural and peripheral tissues) and gestational tissues, parasites, insects, plants, compost/soil mixtures, and swabs from trash containers. Importantly, these results helped to identify seeding-competent prions in places reported to be free of CWD. The levels of prion infectivity in most of these samples are currently being investigated. Conclusions: Our findings contribute to the understanding of the transmission dynamics and prevalence of CWD. In addition, our data have helped to identify CWD in areas previously considered to be free of CWD. We also demonstrate that PMCA is a powerful technique for the screening of biological and environmental samples. Overall, our research suggests that PMCA may be a useful tool to implement for the surveillance and management of CWD. Funded by: NIH/NIAID and USDA Grant number: 1R01AI132695 (NIH) and AP20VSSPRS00C143 (USDA)

Nasal bot: an emerging vector for natural chronic wasting disease transmission

Paulina Sotoa,b, Francisca Bravo-Risia,b, Carlos Kramma, Nelson Pereza, Rebeca Benaventea, J. Hunter Reedc, Mitch Lockwoodc, Tracy A. Nicholsd, 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 Park and Wildlife Department, Texas, USA; dVeterinary Services Cervid Health Program, United States Department of Agriculture, Animal and Plant Health Inspection Service, Fort Collins, Colorado, USA

Chronic wasting disease (CWD) is a fatal neurodegenerative disease that affects farmed and free-ranging cervids populations. The spread of CWD in cervids is thought to occur through the direct contact between cervids or through the exposure of naïve animals to contaminated environments. Parasites are known vectors of multiple diseases in animals. However, the potential role of parasites in CWD transmission remains unclear. Aims: The main objective of this study was to determine if CWD prions could be detected in the larvae of deer nasal bot flies, a common deer parasite, taken from CWD-infected white-tailed deer (Odocoileus virginianus). Methods: Bot fly larvae were collected from the nasal cavity of naturally infected CWD- positive or CWD non-detect white-tailed deer. The CWD seeding activity of the larvae was interrogated by PMCA. Prion infectivity was also evaluated in cervidized transgenic mouse bioassay (intra-cerebral administration in Tg1536 mice). Mice inoculated with bot larvae homogenate were sacrificed when they showed established signs of prion disease, or at extended periods after treatment (600 days). All inoculated mouse brains were evaluated for protease resistant prions to confirm clinical or sub-clinical infection. Bot larvae from CWD non-detect deer were used as controls. To further mimic environmental transmission, bot larvae homogenates were mixed with soils and plants were grown on them. Both plants and soils were tested for prion seeding activity. Results: PMCA analysis demonstrated CWD seeding activity in nasal bot larvae from captive and free-ranging white-tailed deer. CWD-contaminated bots efficiently infected transgenic mice, with attack rates and incubation periods suggesting high infectivity titers. Further analyses of treated animals (biochemical characterization of protease resistant prions and immunohistochemistry) confirmed prion infection. Analyses on dissected parts of the bot larvae demonstrate that the infectivity is concentrated in the larvae cuticle (outer part). Nasal bot larvae extracts mixed with

soils showed seeding activity by PMCA. Interestingly, plants grown in soil contaminated with the nasal bot larvae extract were found to produce seeding activity by PMCA. Conclusion: In this study we described for the first time that deer nasal bot larvae from CWD-infected deer carry high CWD infectivity titers. We also demonstrate that CWD prions in these parasites can interact with other environmental components relevant for disease transmission. Considering this information, we propose that deer nasal bot larvae could act as vectors for CWD transmission in wild and farming settings. Funded by: NIH/NIAID and USDA/APHIS Grant number: R01AI132695 and AP20VSSPRS00C143 PRION 2022 ABSTRACTS, AND A BIG THANK YOU TO On behalf of the Prion2020/2022 Congress Organizing Committee and the NeuroPrion Association, we heartily invite you to join us for the International Conference Prion2020/2022 from 13.-16. September 2022 in Göttingen.

Prion 2022 Conference abstracts: pushing the boundaries

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

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

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

Carrot plants as potential vectors for CWD transmission

Paulina Sotoa,b, Francisca Bravo-Risia,b, Claudio Sotoa, 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

Prion diseases are infectious neurodegenerative disorders afflicting humans and other mammals. These diseases are generated by the misfolding of the cellular prion protein into a disease-causing isoform. Chronic wasting disease (CWD) is a prevalent prion disease affecting cervids (captive and free-range). CWD is thought to be transmitted through direct animal contact or by indirect exposure to contaminated environments. Many studies have shown that infectious prions can enter the environment through saliva, feces, or urine from infected animals and decaying carcasses. However, we do not fully understand the specific contribution of each component to disease transmission events. Plants are logical environmental components to be evaluated since they grow in environments contaminated with CWD prions and are relevant for animal and human nutrition.

Aims: The main objective of this study is to study whether prions are transported to the roots and leaves of carrots, an edible plant commonly used in the human diet and as deer bait.

Methods: We have grown carrot plants in CWD-infected soils. After 90 days, we harvested the carrots and separated them from the leaves. The experiment was controlled by growing plants in soil samples treated with brain extracts from healthy animals. These materials were interrogated for their prion seeding activity using the Protein Misfolding Cyclic Amplification (PMCA) technique. Infectivity was evaluated in mouse bioassays (intracerebral injections in Tg1536 mice). The animals were sacrificed when they showed established signs of prion disease. Animals not displaying clinical signs were sacrificed at 600 days post-inoculation.

Results: The PMCA analysis demonstrated CWD seeding activity in soils contaminated with CWD prions, as well as in carrot plants (leaves and roots) grown on them. Bioassays demonstrated that both leaves and roots contained CWD prions in sufficient quantities to induce disease (92% attack rate). As expected, animals treated with prion-infected soils developed prion disease at shorter incubation periods (and complete attack rates) compared to plant components. Animals treated with soil and plant components exposed with CWD-free brain extracts did not display prion-associated clinical signs or evidence of sub-clinical prion infection.

Conclusions: We show that edible plant components can absorb prions from CWD contaminated soils and transport them to their aerial parts. Our results indicate that plants could participate as vectors of CWD transmission. Importantly, plants designated for human consumption represent a risk of introducing CWD prions into the human food chain.

Funded by: NIH

Grant number: R01AI132695

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies

Location: Virus and Prion Research

Title: Transmission of the atypical/nor98 scrapie agent to suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes

Author item Cassmann, Eric item MAMMADOVA, JAJIBA - Orise Fellow item BENESTAD, SYLVIE - Norwegian Veterinary Institute item MOORE, SARA JO - Orise Fellow item Greenlee, Justin

Submitted to: PLoS ONE Publication Type: Peer Reviewed Journal Publication Acceptance Date: 1/21/2021 Publication Date: 2/11/2021

Citation: Cassmann, E.D., Mammadova, J., Benestad, S., Moore, S., Greenlee, J.J. 2021. Transmission of the atypical/nor98 scrapie agent to suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes. PLoS ONE. 16(2). Article e0246503. https://doi.org/10.1371/journal.pone.0246503. DOI: https://doi.org/10.1371/journal.pone.0246503

Interpretive Summary: Atypical scrapie is a prion disease that affects sheep. Unlike classical scrapie, atypical scrapie is thought to occur spontaneously, and it is unlikely to transmit between sheep under natural conditions. Another notable distinction between classical and atypical scrapie is the prion protein genotype of afflicted sheep and the locations in the brain where misfolded prions accumulate. Atypical scrapie generally occurs in sheep that are resistant to classical scrapie. Misfolded prions are predominantly found in the cerebellum for atypical scrapie and not in the brainstem as seen with classical scrapie. Atypical scrapie is a relevant disease because of its potential association with other prion diseases. Some research has shown that the atypical scrapie agent can undergo a transformation of disease forms that makes it appear like classical scrapie or classical bovine spongiform encephalopathy (mad cow disease). Therefore, atypical scrapie is thought to be a possible source for these prion diseases. We investigated the transmission of the atypical scrapie agent to sheep with three different prion protein genotypes. A diagnosis of atypical scrapie was made in all three genotypes of sheep. Misfolded prion protein was detected earliest in the cerebellum and the retina. This is the first report describing the early accumulation of misfolded prions in the retina of sheep with atypical scrapie. Understanding where misfolded prions accumulate in cases of atypical scrapie can lead to better detection earlier in the disease. Furthermore, the materials derived from this experiment will aid in investigating origins of other prion diseases.

Technical Abstract: Scrapie is a transmissible spongiform encephalopathy that occurs in sheep. Atypical/Nor98 scrapie occurs in sheep with that tend to be resistant to classical scrapie and it is thought to occur spontaneously. The purpose of this study was to test the transmission of the Atypical/Nor98 scrapie agent in three genotypes of Suffolk sheep and characterize the distribution of misfolded prion protein (PrPSc). Ten sheep were intracranially inoculated with brain homogenate from a sheep with Atypical/Nor98 scrapie. All sheep with the ARQ/ARQ and ARQ/ARR genotypes developed Atypical/Nor98 scrapie confirmed by immunohistochemistry, and one (1/3) sheep with the VRQ/ARQ genotype had detectable PrPSc consistent with Atypical/Nor98 scrapie at the experimental endpoint of 8 years. Sheep with mild early accumulations of PrPSc in the cerebellum had concomitant retinal PrPSc. Accordingly, large amounts of retinal PrPSc were identified in clinically affected sheep and sheep with dense accumulations of PrPSc in the cerebellum.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=379280

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Scrapie in white-tailed deer: a strain of the CWD agent that efficiently transmits to sheep?

Author item Greenlee, Justin item KOKEMULLER, ROBYN - US Department Of Agriculture (USDA) item MOORE, S - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, M - Iowa State University

Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 3/29/2019 Publication Date: N/A Citation: N/A

Interpretive Summary:

Technical Abstract: Scrapie is a transmissible spongiform encephalopathy of sheep and goats that is associated with widespread accumulation of abnormal prion protein (PrPSc) in the central nervous and lymphoid tissues. Chronic wasting disease (CWD) is the natural prion disease of cervid species, and the tissue distribution of PrPSc in affected cervids is similar to scrapie in sheep. There are several lines of evidence that suggest that multiple strains of CWD exist, which may affect the agent’s potential to transmit to hosts of the same or different species. We inoculated white-tailed deer with the scrapie agent from ARQ/ARQ sheep, which resulted in 100% attack rates by either the intracranial or oronasal route of inoculation. When examining tissues from the brainstems or lymphoid tissues by traditional diagnostic methods such as immunohistochemistry or western blots, it is difficult to differentiate tissues from deer infected with scrapie from those infected with CWD. However, there are several important differences between tissues from scrapie-infected white-tailed deer (WTD scrapie) and those infected with CWD (WTD CWD). First, there are different patterns of PrPSc deposition in the brains of infected deer: brain tissues from deer with WTD scrapie had predominantly particulate and stellate immunoreactivity whereas those from deer with WTD-CWD had large aggregates and plaque-like staining. Secondly, the incubation periods of WTD scrapie isolates are longer than CWD isolates in mice expressing cervid prion protein. Most notably, the transmission potential of these two isolates back to sheep is distinctly different. Attempts to transmit various CWD isolates to sheep by the oral or oronasal routes have been unsuccessful despite observation periods of up to 7 years. However, WTD scrapie efficiently transmitted back to sheep by the oronasal route. Upon transmission back to sheep, the WTD scrapie isolate exhibited different phenotypic properties when compared to the sheep receiving the original sheep scrapie inoculum including different genotype susceptibilities, distinct PrPSc deposition patterns, and much more rapid incubation periods in transgenic mice expressing the ovine prion protein. The scrapie agent readily transmits between sheep and deer after oronasal exposure. This could confound the identication of CWD strains in deer and the eradication of scrapie from sheep.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=361032

''The scrapie agent readily transmits between sheep and deer after oronasal exposure. This could confound the identication of CWD strains in deer and the eradication of scrapie from sheep.''

RT-QuIC detection of pathological prion protein in subclinical goats following experimental oral transmission of L-type BSE

Alessandra Favole1* , Maria Mazza1 , Antonio D’Angelo2 , Guerino Lombardi3 , Claudia Palmitessa1 , Luana Dell’Atti1 , Giulia Cagnotti2 , Elena Berrone1 , Marina Gallo1 , Tiziana Avanzato1 , Erika Messana1 , Loretta Masoero1 , Pier Luigi Acutis1 , Daniela Meloni1 , Franco Cardone4 , Maria Caramelli1 , Cristina Casalone1 and Cristiano Corona1*

Abstract

Objective: The spread of bovine spongiform encephalopathy (BSE) agent to small ruminants is still a major issue in the surveillance of transmissible spongiform encephalopathies (TSEs). L-type bovine spongiform encephalopathy (L-BSE) is an atypical form of BSE with an unknown zoonotic potential that is transmissible to cattle and small ruminants. Our current knowledge of bovine atypical prion strains in sheep and goat relies only on experimental transmission studies by intracranial inoculation. To assess oral susceptibility of goats to L-BSE, we orally inoculated five goats with cattle L-BSE brain homogenates and investigated pathogenic prion protein (PrPsc) distribution by an ultrasensitive in vitro conversion assay known as Real-Time Quaking Induced Conversion (RT-QuIC).

Results: Despite a prolonged observation period of 80 months, all these animals and the uninfected controls did not develop clinical signs referable to TSEs and tested negative by standard diagnostics. Otherwise, RT-QuIC analysis showed seeding activity in five out of five examined brain samples. PrPsc accumulation was also detected in spinal cord and lymphoreticular system. These results indicate that caprine species are susceptible to L-BSE by oral transmission and that ultrasensitive prion tests deserve consideration to improve the potential of current surveillance systems against otherwise undetectable forms of animal prion infections.

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Discussion and conclusions

Data here presented indicate that caprine species are susceptible to L-BSE after oral administration and are able to produce very low levels of prions in both lymphatic and central nervous tissues as demonstrated by optimized, high-sensitive, RT-QuIC assay.

At variance with goats intracerebrally infected with L-BSE [4], in this study, no animal developed clinical signs of disease despite prolonged periods of observation, suggesting a comparatively low efficiency of the oral route versus the intracerebral one in L-BSE, a feature that further distinguish this strain from classical BSE [14, 15].

Interestingly, all goats tested negative by standard diagnostics for PrPsc performed on brainstem. This finding, associated with the low amount of PrPsc detected in different brain areas, suggests a partial strain-specific transmission barrier. Indeed, inoculation of a prion into a new host species can produce prolonged incubation periods and/or subclinical infection [16, 17]. In addition, the lack of clinical signs suggests that naturally L-BSE-infected goats may be asymptomatic similarly to what proposed by Okada et al. for oral L-BSE in cattle [17].

In line with previous results [18], RT-QuIC detected lower levels of prions than traditional diagnostic tools. Rapid and confirmatory tests failed to identify any PrPsc in the subclinical animals, while RT-QuIC allowed us to detect misfolded prion protein in multiple brain regions, spinal cord and lymphoreticular system. Studies have established that the rate of fluorescence increase in RTQuIC, while not measuring infectivity, is directly related to the concentration of prions in the sample seeding the reaction [19, 20]. Prolonged lag phases of RT-QuIC reactions indicate relatively low amounts of PrPsc in the examined tissues and may reassure about the possibility of goat to play as silent L-BSE spreaders in natural conditions. However, we believe that prudence must be always adopted when dealing with the risk of prion spread in field conditions as also suggested by recent data by Denkers and colleagues, who showed that the oral route of infection for chronic wasting disease in deer, may be much more efficient than previously thought [21]. Furthermore, although the mere presence of PrPsc is not indicative of a possible infectivity of the tissue, the finding of positivity in the lymphoreticular tissue must alert to the potential distribution of PrPsc in peripheral body regions which may increase the risks for humans. Bioassay of infectivity by inoculation of susceptible animals with brains of these goats may help to clarify this issue.

Based on the results achieved with this prion form and also other animal strains, it would be useful to consider the possibility to enlarge current diagnostic criteria to include, in defined conditions (e.g. very limited amounts of source tissue, or preclinical testing), the application of ultrasensitive diagnostic methods. This will not only improve the sensitivity of our surveillance systems but will also help to protect food chain from accidental spillovers of the agent of L-BSE.

Limitations

Te primary limitation of this work is that infectivity was not demonstrated by bioassay and the infectious titre was not determined. Terefore, we cannot comment the degree of risk for human.

Despite these limitations, this work specifcally demonstrates prion-seeding activity in tissues of goats orally exposed to L-BSE and provide RT-QuIC as useful method to enhance surveillance of TSEs.

Keywords: Prion, L-BSE, RT-QuIC, Goat, Oral transmission, PrPsc, Ultrasensitive detection

snip...see full text;

https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC8650279&blobtype=pdf

WEDNESDAY, MARCH 16, 2022

SHEEP BY-PRODUCTS AND WHAT ABOUT Scrapie TSE PrP and Potential Zoonosis?

https://transmissiblespongiformencephalopathy.blogspot.com/2022/03/sheep-by-products-and-what-about.html

SO, WHO'S UP FOR SOME MORE TSE PRION POKER, WHO'S ALL IN $$$

SO, ATYPICAL SCRAPIE ROUGHLY HAS 50 50 CHANCE ATYPICAL SCRAPIE IS CONTAGIOUS, AS NON-CONTAGIOUS, TAKE YOUR PICK, BUT I SAID IT LONG AGO WHEN USDA OIE ET AL MADE ATYPICAL SCRAPIE A LEGAL TRADING COMMODITY, I SAID YOUR PUTTING THE CART BEFORE THE HORSE, AND THAT'S EXACTLY WHAT THEY DID, and it's called in Texas, TEXAS TSE PRION HOLDEM POKER, WHO'S ALL IN $$$

***> AS is considered more likely (subjective probability range 50–66%) that AS is a non-contagious, rather than a contagious, disease.

https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2021.6686

***> All sheep with the ARQ/ARQ and ARQ/ARR genotypes developed Atypical/Nor98 scrapie confirmed by immunohistochemistry, and one sheep with the VRQ/ARQ genotype had detectable PrPSc consistent with Atypical/Nor98 scrapie at the experimental endpoint of 8 years.

Transmission of the atypical/Nor98 scrapie agent to Suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes

Eric D. Cassmann, Najiba Mammadova, S. Jo Moore, Sylvie Benestad, Justin J. Greenlee

Published: February 11, 2021

https://doi.org/10.1371/journal.pone.0246503

Abstract

Scrapie is a transmissible spongiform encephalopathy that occurs in sheep. Atypical/Nor98 scrapie occurs in sheep that tend to be resistant to classical scrapie and it is thought to occur spontaneously. The purpose of this study was to test the transmission of the Atypical/Nor98 scrapie agent in three genotypes of Suffolk sheep and characterize the distribution of misfolded prion protein (PrPSc). Ten sheep were intracranially inoculated with brain homogenate from a sheep with Atypical/Nor98 scrapie. All sheep with the ARQ/ARQ and ARQ/ARR genotypes developed Atypical/Nor98 scrapie confirmed by immunohistochemistry, and one sheep with the VRQ/ARQ genotype had detectable PrPSc consistent with Atypical/Nor98 scrapie at the experimental endpoint of 8 years. Sheep with mild early accumulations of PrPSc in the cerebellum had concomitant retinal PrPSc. Accordingly, large amounts of retinal PrPSc were identified in clinically affected sheep and sheep with dense accumulations of PrPSc in the cerebellum.

https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0246503

***> These data confirm that ARR/ARR sheep cannot be considered to be fully resistant to classical scrapie.

Journal of General Virology header logoVolume 98, Issue 8

Classical scrapie transmission in ARR/ARR genotype sheep Free

Caroline Lacroux1,†, Hervé Cassard1,†, Hugh Simmons2, Jean Yves Douet1, Fabien Corbière1, Severine Lugan1, Pierette Costes1, Naima Aron1, Alvina Huor1, Cécile Tillier1, Francois Schelcher1, Olivier Andreoletti1

Published: 01 August 2017 https://doi.org/10.1099/jgv.0.000861 ;

ABSTRACT

The ARR allele is considered to provide a very strong resistance against classical scrapie infection in sheep. In this study, we report the occurrence of clinical transmissible spongiform encephalopathy in ARR/ARR sheep, following their inoculation by the intracerebral route with a classical scrapie isolate. On first passage, the disease displayed an incomplete attack rate transmission, with incubation periods exceeding 6 years. On second passage, the obtained prion did not display better abilities to propagate than the original isolate. These transmission results contrasted with the 100 % attack rate and the short incubation periods observed in ARQ/ARQ sheep challenged with the same isolate. These data confirm that ARR/ARR sheep cannot be considered to be fully resistant to classical scrapie. However, they also support the contention that classical scrapie has a very limited capacity to transmit and adapt to ARR/ARR sheep.

https://www.microbiologyresearch.org/content/journal/jgv/10.1099/jgv.0.000861#tab2

''These data confirm that ARR/ARR sheep cannot be considered to be fully resistant to classical scrapie. However, they also support the contention that classical scrapie has a very limited capacity to transmit and adapt to ARR/ARR sheep.''

Emerg Infect Dis. 2007 Aug; 13(8): 1201–1207.

doi: 10.3201/eid1308.070077

PMCID: PMC2828083

PMID: 17953092

Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France

Martin H. Groschup,corresponding author* 1 Caroline Lacroux,† 1 Anne Buschmann,* Gesine Lühken,‡ Jacinthe Mathey,† Martin Eiden,* Séverine Lugan,† Christine Hoffmann,* Juan Carlos Espinosa,§ Thierry Baron,¶ Juan Maria Torres,§ Georg Erhardt,‡ and Olivier Andreoletti†

Abstract

In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A136R154R171 haplotype of the prion protein. This genotype was therefore assumed to confer resistance to BSE and classic scrapie under natural exposure conditions. Hence, to exclude prions from the human food chain, massive breeding efforts have been undertaken in the European Union to amplify this gene. We report the identification of 2 natural scrapie cases in ARR/ARR sheep that have biochemical and transmission characteristics similar to cases of classic scrapie, although the abnormally folded prion protein (PrPSc) was associated with a lower proteinase-K resistance. PrPSc was clearly distinct from BSE prions passaged in sheep and from atypical scrapie prions. These findings strongly support the idea that scrapie prions are a mosaic of agents, which harbor different biologic properties, rather than a unique entity.

snip...

The discovery of these 2 cases clearly indicates that the genetic resistance of ARR/ARR sheep to the so-called classic scrapie agent is not absolute. It also provides evidence that, rather than being a single entity, scrapie is a mosaic of infectious agents harboring different biologic properties in its natural host. Finally, although many thousands of cases of classic scrapie have been reported in sheep of other PrP genotypes and hundreds of thousands of rapid tests have been performed in Europe since the implementation of active TSE surveillance in small ruminants began in 2001, the discovery of these 2 ARR/ARR cases supports the idea that such infections are extremely rare.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2828083/pdf/07-0077_finalR.pdf

''The discovery of these 2 cases clearly indicates that the genetic resistance of ARR/ARR sheep to the so-called classic scrapie agent is not absolute.''

Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice.

*** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,? +Author Affiliations

*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

***Edited by Stanley B. Prusiner, University of California, San Francisco, CA (received for review March 21, 2005)

Abstract

Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. *** These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

http://www.pnas.org/content/102/44/16031.abstract

''These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.''

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 ===============

https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html

''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

https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html

***> ''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) <***

https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html

***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.

https://www.nature.com/articles/srep11573

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.

==============

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019499/

***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.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

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.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

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.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

GAME FARM INDUSTRY WANTS TO COVER UP FINDINGS OF INCREASE RISK TO CJD FROM CERVID

BSE INQUIRY

CJD9/10022

October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane

BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended.. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

The statistical results regarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasized by the finding that some strains of scrapie produce lesions identical to the once which characterize the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the scrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

reference...

RB3.20

TRANSMISSION TO CHIMPANZEES

1. Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.

2. We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, ilp and i/v) :

3. I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. Proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.

4. In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.

5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

6. A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans' susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday' s meeting.

R. Bradley

23 September 1990

CVO (+Mr Wells' comments)

Dr T W A Little

Dr B J Shreeve

90/9.23/1.1.

http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf

IN CONFIDENCE CHIMPANZEES

CODE 18-77 Reference RB3.46

Some further information that may assist in decision making has been gained by discussion with Dr Rosalind Ridley.

She says that careful study of Gajdusek's work shows no increased susceptibility of chimpanzees over New World Monkeys such as Squirrel Monkeys. She does not think it would tell you anything about the susceptibility to man. Also Gajdusek did not, she believes, challenge chimpanzees with scrapie as severely as we did pigs and we know little of that source of scrapie. Comparisons would be difficult. She also would not expect the Home Office to sanction such experiments here unless there was a very clear and important objective that would be important for human health protection. She doubted such a case could be made. If this is the case she thought it would be unethical to do an experiment abroad because we could not do it in our own country.

Retrospectively she feels they should have put up more marmosets than they did. They all remain healthy. They would normally regard the transmission as negative if no disease resulted in five years.

We are not being asked for a decision but I think that before we made one we should gain as much knowledge as we can. If we decided to proceed we would have to bear any criticisms for many years if there was an adverse view by scientists or media. This should not be undertaken lightly. There is already some adverse comment here, I gather, on the pig experiment though that will subside.

The Gibbs' (as' distinct from Schellekers') study is somewhat different. We are merely supplying material for comparative studies in a laboratory with the greatest experience of human SEs in the world and it has been sanctioned by USDA (though we do not know for certain yet if chimpanzees specifically will be used). This would keep it at a lower profile than if we conducted such an experiment in the UK or Europe.

I consider we must have very powerful and defendable objectives to go beyond Gibbs' proposed experiments and should not initiate others just because an offer has been made.

Scientists have a responsibility to seek other methods of investigative research other than animal experimentation. At present no objective has convinced me we need to do research using Chimpanzees - a species in need of protection. Resisting such proposals would enable us to communicate that information to the scientist and the public should the need arise. A line would have been drawn.

CVO cc Dr T Dr B W A Little Dr B J Shreeve

R Bradley

26 September 1990

90/9.26/3.2

http://web.archive.org/web/20090506041605/http://www.bseinquiry.gov.uk/files/yb/1990/09/26003001.pdf

this is tse prion political theater here, i.e. what i call TSE PRION POKER...tss

http://web.archive.org/web/20031028205208/www.bseinquiry.gov.uk/files/yb/1990/08/28002001.pdf

http://web.archive.org/web/20030822170317/www.bseinquiry.gov.uk/files/yb/1990/11/01005001.pdf

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.

snip...

PAGE 26

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.

The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province! ...page 26.

snip...see;

IN CONFIDENCE

PERCEPTIONS OF UNCONVENTIONAL SLOW VIRUS DISEASE OF ANIMALS IN THE USA

GAH WELLS

REPORT OF A VISIT TO THE USA

APRIL-MAY 1989

http://web.archive.org/web/20090506002237/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man.

***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough.

***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

snip...

https://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf

SATURDAY, SEPTEMBER 24, 2022

Transmission of CH1641 in cattle

https://transmissiblespongiformencephalopathy.blogspot.com/2022/09/transmission-of-ch1641-in-cattle.html

Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission

https://www.regulations.gov/comment/APHIS-2021-0004-0002

https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf

Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification

https://www.regulations.gov/document/APHIS-2018-0011-0003

https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf

Sunday, January 10, 2021

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission June 17, 2019

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission

Greetings APHIS et al,

I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.

THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal.

Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban.

The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products 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. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.

WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.

WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.

AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...

https://www.regulations.gov/comment/APHIS-2018-0087-0002

https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf

http://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.230.8886&rep=rep1&type=pdf

PRION 2022 ABSTRACTS, AND A BIG THANK YOU TO

On behalf of the Prion2020/2022 Congress Organizing Committee and the NeuroPrion Association, we heartily invite you to join us for the International Conference Prion2020/2022 from 13.-16. September 2022 in Göttingen.

Prion 2022 Conference abstracts: pushing the boundaries

https://www.tandfonline.com/doi/full/10.1080/19336896.2022.2091286

https://prionconference.blogspot.com/2022/09/prion-conference-2022-abstracts-cwd-tse.html

snip...see full text;

SUNDAY, OCTOBER 16, 2022

USDA Transmissible Spongiform Encephalopathy TSE Prion Action Plan National Program 103 Animal Health 2022-2027

https://transmissiblespongiformencephalopathy.blogspot.com/2022/10/usda-transmissible-spongiform.html

SATURDAY, OCTOBER 8, 2022

Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation

https://bovineprp.blogspot.com/2022/10/cattle-with-ek211-prnp-polymorphism-are.html

MONDAY, AUGUST 29, 2022

Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies 2021 Annual Report

https://transmissiblespongiformencephalopathy.blogspot.com/2022/08/pathobiology-genetics-and-detection-of.html

FRIDAY, SEPTEMBER 23, 2022

SPILLOVER CWD TSE PRION INTO DIFFERENT SPECIES, pigs, sheep, cattle, camel, and humans, what if?

https://chronic-wasting-disease.blogspot.com/2022/09/spillover-cwd-tse-prion-into-different.html

SUNDAY, OCTOBER 16, 2022 USDA

Transmissible Spongiform Encephalopathy TSE Prion Action Plan National Program 103 Animal Health 2022-2027

https://transmissiblespongiformencephalopathy.blogspot.com/2022/10/usda-transmissible-spongiform.html

TUESDAY, OCTOBER 18, 2022

Assessing the Potential Transmissibility of Bovine and Cervid Prions with a Human Prion Protein-based Model ARS RESEARCH