CHRONIC WASTING DISEASE CWD TSE PRION ZOONOSIS ZOONOTIC INSIDIOUS AND DIRE CONSEQUENCES AHEAD
Alliance for Public Wildlife Living Legacy White Paper
The Challenge of CWD: Insidious and Dire Only immediate action will avoid catastrophic outcomes
Valerius Geist, Professor Emeritus, University of Calgary David Clausen, (former) Chair, Wisconsin Natural Resources Board Vince Crichton, (former) Co-Chair, Canada’s National Wildlife Disease Strategy Darrel Rowledge, Director, Alliance for Public Wildlife
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CWD is now deemed to be the largest-ever mass of infectious prions in global history, and experts sum up the threat (to wildlife, agriculture, our economies, and potentially to human health) in two words: “insidious and dire.” Current policy and apathy toward the levels of CWD consumption by people has been described as “one of the most outrageous human susceptibility experiments in history.”
We have a problem. A big problem. Chronic Wasting Disease (CWD), a sister to BSE or ‘mad cow,’ is threatening our deer and elk. Unfortunately, CWD has broad implications. Without immediate action, we are heading for worst cases outcomes that include severe population impacts, extinctions, crashing economies, and, although unlikely, potential transfers of CWD to people.
Chronic Wasting Disease is an incurable, always fatal degeneration of the brain. Technically, it’s a Transmissible Spongiform Encephalopathy (TSE), but there are a number of quite different versions, depending on species. They include in humans kuru and fatal familial insomnia, as well as some with even more unpronounceable names, such as the dreadful human Creutzfeldt-Jakob Disease (CJD) and Gerstmann–Sträussler–Scheinker Disease (GSS). The largest TSE epidemics have been in domestic or captive animals: such as Scrapie in domestic sheep, Bovine Spongiform Encephalopathy (BSE), or so-called ‘mad cow’ disease, Transmissible Mink Encephalopathy (TME) on mink farms, and CWD in captive deer and elk.
CWD emerged as a particular nasty variant, because it can be transmitted by body fluids of infected animals (urine, feces, and saliva). Unlike BSE, CWD is highly contagious and can spread to and through wild ungulate herds. The infective agents are mis-folded proteins called prions; they are virtually indestructible, can persist in the environment, and tiny quantities can transmit the disease. Prion diseases have repeatedly jumped species barriers—most alarmingly in the United Kingdom, when BSE-infected beef killed 229 people.
As CWD spread, naturally and through trade, the U.S. in 2001 officially declared a “State of Emergency.” Every factor has since gotten worse. It has now been confirmed in 24 US states, 3 Canadian provinces, South Korea, and recently in Norway. Field studies are confirming potentially severe impacts on wildlife populations. So far no transmission to humans has been documented, but the risk is not zero. Non-human primates and transgenic (humanized) mice have been infected. In many jurisdictions, a lack of awareness and availability of free, rapid, and convenient testing of harvested deer has led to significant level of human exposure. Estimates show 7,000 to 15,000 CWD-infected animals are being consumed by hunter families every year, and this number continuing to rise by as much as 20% per year. The combination of threats is sobering. CWD has been shown to persist and remain infectious in the environment, including in clay-based soils that can dramatically increase infectivity (up to 680 times). Decomposing carcasses create contaminated “super-sites.” Prions are extremely resilient, known to resist disinfectants, alcohol, formaldehyde, detergents, protein enzymes, desiccation, radiation, freezing, and incineration >1100°F. Facilities infected with CWD have resisted all efforts at removing the infective agent. Canadian officials report that even on premises thought to be very low risk, restocking with healthy animals led to a 50% re-occurrence of CWD.
Transmission occurs animal to animal, soil to animal, mother-to-offspring, and from exposed plants or other surfaces including tools or surgical instruments (even autoclaving is ineffective). Now there is evidence the infective agent is taken up via the root systems of plants growing in contaminated soils, with transfer to stems and leaves. These were shown to be infective via inter-cerebral injection (oral tests are ongoing).
Executive Summary
Left unchecked, the prospects for wildlife are bleak. CWD has clear population impacts; some models suggest extinction. Disproportionate impact on mature males carries implications for hunters and wildlife economies let alone populations. Still more bad news: Efforts for vaccines have failed, and evolutionary or adaptive salvation is unlikely and would be too late in any case. CWD is now deemed to be the largest-ever mass of infectious prions in global history, and experts sum up the threat (to wildlife, agriculture, our economies, and potentially to human health) in two words: “insidious and dire.” Current policy and apathy toward the levels of CWD consumption by people has been described as “one of the most outrageous human susceptibility experiments in history.”
The good news
There is, of course, much more—but we need to get to the good news: There is hope, beginning with the fact that CWD is relatively new—not a long-standing or indigenous disease of our wildlife. The vast majority of our herds are still disease-free. We have considerable expertise, leading-edge technologies, and the benefit of experience. We faced a crisis on this scale once before, almost exactly a century ago, when the very existence of wildlife on this continent was threatened by the severest of over-exploitation. Hunters and conservation organizations led the efforts to avert disaster. With the courage and foresight of presidents and prime ministers enlisting the best and ablest on both sides of the US/Canada border to enact science-based policies, they turned our greatest tragedy into a ‘triumph of the commons.’ Anchored in the public trust doctrine, and now recognized as the North American Model of Wildlife Conservation, it replenished an entire continent with wildlife.
We need, today, nothing less than a similar effort to manage the Chronic Wasting Disease crisis. We have the benefit of experience and principles for success. Following the Roosevelt Doctrine, the same concerned hunter and conservation organizations must once again be the standard-bearers of principled, science-and evidence-based leadership in wildlife conservation. We must be relentless in following the leading science and scholarship, tracking the evidence, and engaging in comprehensive analysis to foresee the implications. We understand how policies affect the spread of diseases, as documented in the scientific and historical record summarized below. This threat is dire, and immediate action is warranted.
While details and methods must be guided by science and evidence, there is significant agreement on critical needs; and we have assurances from leading experts and labs that we have the capacity to meet this challenge. We must secure mandate and funding to:
1. Contain the geographic spread of CWD by enacting and enforcing an immediate ban on the movement of all live cervids, all potentially CWD-infected carcasses, animal parts, products, exposed equipment, trailers, or other sources of infectious materials.
2. Mandate and implement for hunters, convenient, cost-free, rapid testing of all animals harvested from CWD-affected areas.
3. Ensure that no CWD-infected material reaches the food or feed chains, and that it is instead properly disposed of.
4. Establish and fund accountable research and science-based policy to protect public interest (health, wildlife and related industries, agriculture, our economies and communities).
The issues are numerous, serious, and complex, but complacency is not an option. The sooner we act, the greater the prospects to protect our greatest living legacy. Further details, discussion, citations, and scientific references follow.
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Potential Risk of Transfer to People There are few considerations which require greater foundational context than questions of zoonotic risk of infectious diseases. Combining public policy and science is very much a matter of addressing uncertain risks that are dynamic, evolving, and with complex, even profound consequences.
The reality is that most (~70%) emerging zoonotic diseases have come from animals.150, 151 Each presented uncertain risks, and in every instance there was a point in history where the animal to human transfer of that particular pathogen had not yet occurred. Such absence of evidence or ‘proof’ can often elicit false inferences that dismiss or underestimate the risk. The UK example of BSE (unexpectedly) transferring to people as vCJD, is but one recent example.152 The impacts were complex, extending far beyond immediate victims, bringing serious and prolonged socioeconomic and health consequences that included suicides tied to the severe economic impacts of BSE on the agricultural economy.153 There are persisting uncertain zoonotic risks related to the BSE that remain to this day. For example, findings in lymphoreticular tissue (archived through appendix samples) indicate that 1 in 2,000 of the UK population are asymptomatic carriers infected with abnormal PrP.154 The long term implications are unknown.
Zoonotic risks are neither static nor merely historic phenomena: “it is estimated that approximately 75 per cent of ‘new’ human pathogens reported in the past 25 years have originated in animals and the risk of zoonoses is predicted to continue to increase.”155 As status quo matters of public policy they require consideration of known and potential consequences. “The Global Burden of Disease Study estimates that, in the year 2000, infectious diseases were responsible for 22% of all deaths and 27% of disability-adjusted life years worldwide.”156
Such risk profiles can only be considered as snapshots in dynamic, evolving landscapes, where observation and evidence of variability—indicating change or evolution—is a vital consideration. This underscores the very essence of the precautionary principle, and nowhere is it more requisite than with respect to infectious pathogens. Inadequate policy or regulatory failures can result in pandemics that kill thousands or even millions of people or other animals, causing enormous damage on economies and ecosystems.
The Precautionary Principle
Where there is a potential for severe or irreversible harm, especially to public wellbeing and interest, an absence of scientific consensus or proof of harm cannot be used to allow or maintain policies or actions underlying the risk. In such cases, the burden to ‘prove safety’ falls on those advocating the potentially harmful policy or action.157
The standard of “severe or irreversible harm” is a very high bar; yet one CWD has long surpassed regarding public wildlife. It is only against that backdrop that the potential transference of CWD to people can be reasonably considered. We must consider risk, consequences, and even worst case scenarios. The fact is that prion diseases are described by physicians and victim’s families as aggressive, horrific, and dreadful.
Faced, in his medical practice, with the reality of human prion and neurodegenerative diseases, a leading scientists like Dr. Neil Cashman, (former) Scientific Director, PrioNet Canada, have long warned that CWD is “an emergency in slow motion.” At the “On The Horizon” PrioNet Research Conference Dr. Cashman summarized the background and urgency as follows:
“CWD is spreading like wildfire. From a few foci in Saskatchewan, it has now come to involve deer and elk in Alberta and Saskatchewan and there are no geographical barriers. It will spread until it infects the entire continent. It also spreads across species. ...It can persist in water; it can persist in soil. It’s spreading without check. It’s arguably the most contagious prion disease, and the human health impact is unknown. We just frankly do not know if humans are susceptible to chronic wasting disease. It’s an emergency in slow motion.”158
This combination of growth, spread, changing risk, and extreme consequence explains the near unanimity of caution in available zoonotic analyses: “Although the zoonotic potential of CWD is considered low, identification of multiple CWD strains and the potential for agent evolution upon serial passage hinders a definitive conclusion.”159
Assessing zoonotic risk of new, emerging, and especially fatal diseases, is challenged by the inability to experimentally test susceptibility in people. Indeed, the ethical challenges are formidable enough regarding potential treatments. Yet questions of susceptibility require new approaches combining epidemiological and laboratory analyses (both in vitro and in vivo), as well as considerations of known and probable human exposure. Questions of appropriate policy and regulatory responses must be weighed against all implied consequences (biological, social, and economic), and the entire range of outcomes. This must include potential worst case scenarios even if they are thought extremely unlikely, not just because of evolving risk, but because market, media, and societal responses are often based more on perception than on science or reality.
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Experts weigh in
Given their own and the risk analyses of others, leading scientists are expressing concern: “The increasing levels of CWD exposure are highly concerning.” “As a matter of policy, I believe all animals taken from CWD-infected areas should be tested before consumption and people should definitely not be consuming any infected material.”181
Qingzhong Kong, PhD, Case Western Reserve University
“The CWD situation and increasing levels of CWD exposure is a concern for cervid and human public health.” “CWD-testing should be conducted on animals harvested from CWD-infected areas prior to consumption.”182
Candace Mathiason, PhD, Colorado State University
“ … The more opportunity to expose humans to this stuff, the more we’re potentially playing with fire, in terms of these strain adaptations. It wouldn’t take very many cases of human prion disease that were linked back to chronic wasting disease, to where this whole conversation could change, fairly dramatically, and pretty much overnight. So I think while we have the opportunity, to get out in front of this … where we can as best we can, we should probably take advantage of that.”183
Michael Miller, PhD, CO Division of Wildlife
The full spectrum
The scope of human exposure to CWD is broader than is generally appreciated. It includes some direct exposures that have been largely ignored, lessons from history notwithstanding. When early suspicions of BSE being spread through consumption of blood, bone, and nerve tissues were confirmed in 1988,184 it led to bans on feeding meat and bone meal (MBM) supplements.185 Yet the potential risk of CWD in velvet antlers (i.e., blood, bone, and nerve tissue) sold for human consumption continued to be ignored long after both the confirmation of BSE being transferred to people as vCJD,186 and the repeated findings of CWD on game farms.187
A Risk Assessment of TSE products (dated June 2000) undertaken for Health Canada identified “pharmaceutical products containing high risk tissues and elk antler velvet food supplement” as the highest ranking risks.188 Under public pressure the Canadian Food Inspection Agency pledged in October 2000 to destroy velvet antler from CWD-infected animals. However, no recalls nor warnings to potential consumers have ever been issued.189 The difficulty of recalling product widely distributed throughout Asia is accepted; but it also illustrates the challenge and the consequences of failing to detecting potential zoonotic transfer. Furthermore, this passive approach regarding velvet antler continues even after confirmation of PrPd in velvet antler in 2009.190
Why worry?
As evident from challenges in achieving a CWD vaccine for cervids, there is little hope that breakthrough treatments would soon emerge. CWD has been in the shadows; but the toll of other protein misfolding diseases in people (Alzheimer’s, Parkinson’s, Huntington’s, ALS, CreutzfeldtJakob, etc.) affects tens of millions of Americans and cost hundreds of $billions per year. Yet the scale of complexity and level of difficulty is such, that, even after decades of research, there are no cures, and few effective treatments for any of them.191 Moreover, and as with any disease, containing and managing risk of CWD will demand an understanding and acceptance of the relentless capacity of this disease, as it continues to grow, spread, persist, and evolve. “Prions are distinguished from other amyloid diseases both by their infectious character and the observed exponential growth of infectious material.”192
With its growth and spread, human exposure from all sources has been increasing exponentially, and we would do well to consider the implications of both known and unknown factors. For example, prion load has been shown to be relevant, but note the title of McLean and Fryer’s 2011 work “There is No Safe Dose of Prions.” Analysis of 4,338 mice showed “that infection is possible at the very low dose of a 1000-fold dilution of the dose that infects half the challenged animals (ID50).” 193
After pointing out that bank voles (which are circumpolar) are described as the ‘universal acceptor for prions,’ Sigurdson asks if the sequence in the human β2-α2 loop creates a permissive host PrPC sequence that is converted by prions from other species, despite sequence mismatches.194 With multiple avenues of direct and indirect human exposure, potential bioaccumulation, the potential role of co-factors, stressors, and potentially consequential passage to or through intermediate species, caution remains prudent. Moreover, it’s becoming clear that our understanding will be well served by looking beyond mammals. Quite apart from the work documenting mammalian prions taken up or adhering to plants, Susan Lindquist’s team has recently shown the “first protein from the plant kingdom with bona fide prion attributes.”195
Science, our greatest ally
These analyses outline more than risk: they offer hope. Lindquist has long been at the front of breakthrough prion research with yeast, and has not only documented many collaborative interactions, but key evolutionary and epigenetic analyses to help explain the phenotypic benefits that have conserved prion existence for 800 million years. These and other insights 196 into prion function may well open opportunities to prevent, limit, or potentially even reverse prion disease.197
However hopeful those breakthroughs might be, they are distant, and the levels of human exposure to CWD are already into the UK’s range of 1,000—10,000 BSEinfected carcasses sufficient to result in BSE transferring to a person.198 North American hunter families are consuming some 7,000—15,000 CWD-infected animals per year, and the number is growing exponentially.199 Though deer are much smaller in mass than cattle, this is more than offset by the fact that CWD prions are spread far more broadly than BSE prions in tissues most likely to be consumed.200 Prion load per animal may thus be higher in deer, despite the difference in mass. And while retail markets for beef reach more genetically susceptible consumers, that is less comforting when considering that the entire deer is often consumed by one hunter family.
Overall, the zoonotic risk profile of CWD is complex, uncertain and evolving. Without exception, dozens of experts consulted for this work concur with the current Director, Prion Diseases Program for the Public Health Agency of Canada, Dr. Michael Coulthart, who describes the risk of CWD transferring to people as “far from negligible.”201
Implications are broad, deep, and longterm
What is clear to policy analysts is that even a single transfer of CWD to a person will carry catastrophic implications in reactions of the public, in markets, and in public policy and international trade, regardless of how the disease manifests. And we cannot ignore the reality that CWD is highly contagious in deer.202
It is not inconceivable that a possible transfer to people could result in similar prion shedding in urine, feces, and saliva. Such an occurrence is beyond any known, practical means of containment or treatment, or avenues to curtail the economic fallout. If less dramatic, the risk profile is broad, complex, and with potentially dire outcomes at every turn. As to one minor example: consider the risk of CWD transfer through pet food. That was included in the UK’s updated, March 2016 Qualitative Risk Assessment regarding chronic wasting disease being introduced into Great Britain.203 The assessment asks: What is the risk of CWD being introduced into Great Britain (GB) from North America and causing infection in deer?
The analysis focuses on three routes of potential CWD introduction:
1. importation of animal feed
2. importation of deer urine lures
3. importation of CWD prion on contaminated equipment and clothing/footwear of hunters or other tourists and British servicemen
The assessment cites the European Union Trade Control and Expert System (TRACES), which confirmed that “in November and December 2015, for example, GB imported 13.6112 tonnes of processed cat and dog food (including dog chews) containing products of ungulate origin from Canada and USA.”
The UK Assessment points out that while the U.S. Food and Drug Administration (FDA) recommends that CWD positive deer or elk, or even such considered at risk, may not enter the animal feed system, it is only a recommendation. They therefore conclude that CWD risk material “may constitute a small percentage of the very low tonnage of non-fish origin processed animal proteins imported from the U.S. into GB.” therefore considers that “there is a greater than negligible risk that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.” (Emphasis in the original.)
As more information becomes known, and major market players get involved, that status quo is unlikely to stay. Pet food regularly includes a variety of rendered animals and animal parts, including road kills. Sept. 15, 2003 the FDA issued guidance that “Material from CWD positive deer and elk may not be used in any animal feed.” That may have seemed a solid precautionary measure, however, Dr. Dave Clausen (from the CWD positive state of Wisconsin) explained that the results soon turned perverse: “Since results of CWD tests would typically take a few days or weeks, renderers in the CWD areas cite this section as the reason to not accept any deer carcasses that have been tested for CWD. Fear was that a positive test would compromise their interest and/or shut down operation. Thus renderers will take untested carcasses just not tested ones.”204
Then, with CWD continuing to extend its range to 24 states and 2 provinces, the FDA recently took further steps, adding a section to cover: “deer and elk considered at high risk for CWD”205 But as the UK Assessment pointed out, this is mere guidance, and does not establish legally enforceable responsibilities.206 Moreover, FDA rules and guidelines for ensuring composition requirements, compliance, monitoring and enforcement are weak, and interstate and international transport of pet foods is widespread and poorly regulated.207
Given their experience with BSE, the UK assessment of risk via pet food is somewhat surprising. Their experience included the world’s first documented instance of interspecies transfer of prion diseases in 1990, when a house cat developed scrapie-like skin irritation so fierce he licked himself bare. Dubbed “Mad Max” by the British press, the cat died from Feline Spongiform Encephalopathy (FSE), a toll eventually reaching 89 domestic cats in UK, one in Northern Ireland, one in Norway, one in Switzerland, and one in Liechtenstein.
Virtually all species of large cats in zoos were similarly infected, including: five cheetahs, three pumas, three ocelots, three tigers, five lions, and one Asian Leopard Cat.208 All were instances of simple oral ingestion of BSEcontaminated feed. The news of transfer across species barriers immediately rocked public confidence and affected markets, which were damaged further with the subsequent admission that people were dying of vCJD from consuming infected beef.209
The UK assessment outlines a similarly “greater than negligible” risk of importation of CWD prion on contaminated equipment and clothing/footwear of hunters, and that “the annual risk of at least one infection of deer in the UK with CWD from deer urine lures imported from the USA is medium.” But whereas official assessments of CWD threats to European wildlife have been measured, largely unseen, and potentially understated, concerns raised by NGOs and the media have been blunt, as in an article in The Times headlined: “Disease from the U.S. could wipe out all the deer in Britain.”210
No known ‘off switch’
Any news of international transfer of CWD by any means, to any species whether deer, rodents, pets, or livestock (such as domestic sheep), would almost certainly have severe consequences. The economic implications would be felt most acutely in North American CWD affected areas.
The UK experience with vCJD is instructive, but key differences are noteworthy. Despite recent confirmation of PrPd in saliva of BSE cattle,211 the absence of (efficient) lateral transfer of BSE between living animals limited growth and spread of the disease,212 and it allowed the ruminant feed ban to eventually halt both the epidemic and the subsequent trade embargo. This underscores a contrast of some significance: CWD with its prolific prion shedding in saliva, feces and urine, is a highly contagious, extremely persistent disease, which has established unprecedented reservoirs in public wildlife and the environment. Compared to BSE, CWD offers no apparent ‘off switch.’
Lessons from BSE
From a public policy perspective, the experience with BSE offers vital lessons—from the foundational frame to the conclusions and recommendations by the official inquiry (paraphrased for brevity):
• “At the heart of the BSE story lie questions of how to handle hazard — a known hazard to cattle and an unknown hazard to humans.”
• “BSE developed into an epidemic as a consequence of intensive farming practice(s) … unchallenged over decades, (that) proved a recipe for disaster.”
• “Government was preoccupied with preventing an alarmist over-reaction … (they) believed that the risk was remote. It is now clear that this campaign of reassurance was a mistake.”
• “Public was repeatedly reassured that it was safe to eat beef.” 213
• “Repeated statements that ‘there is no evidence that BSE is transmissible to humans’ does not explain that such evidence would take many years to emerge.”214
• “Even when risk to humans seems remote, all reasonable precautions must be taken.”
• “There should be more checks on possible pathways of transmission, and on occupational risks.”215
• “Where there is uncertainty, government must not shrink from saying “we are not sure.”216
The analyses and recommendations of the UK experience with BSE are founded on failures of governments to uphold public trust and the precautionary principle. The lessons are directly applicable to CWD: Without immediate, science-based intervention by North American governments to contain and limit the spread, growth, evolution, and exposure of CWD, the likelihood of ‘worstcase’ outcomes will continue to increase, and wildlife is in the cross hairs in every scenario.
Letter of concern regarding CWD from Johnny Morris, Founder/CEO, Bass Pro Shops. (SEE URL LINK BELOW TO READ LETTER...TSS)
It has come to my attention that during the legislative session the General Assembly passed two omnibus agriculture bills (House Bill 1326 and Senate Bill 506) that attempt to redefine the term “livestock” in Missouri statues to include captive deer and put them into the same classification as cattle, sheep and chickens.
I understand that Governor Nixon vetoed the Bill from a constitutional perspective as the Missouri Constitution gives the Conservation Commission authority over the management and control of all game and wildlife resources of the State.
White-tailed deer are wildlife, regardless of whether they are held in captivity or are free-ranging.
The same is true of other wildlife species held in captivity, such as quail, black bear, mountain lions, timber rattlesnakes, raccoons, and squirrels.
Hunting, fishing and outdoor recreation have a significant positive impact on our state’s economy and quality of life.
Over half a million citizens go deer hunting every fall throughout Missouri.
This has a positive economic impact on our state’s economy and many small businesses.
Deer hunting in Missouri generates over $1 billion dollars of business activity annually.
That activity results in over $95 million dollars in state and local tax revenue each year.
A major loss to the white-tailed deer population in Missouri would be devastating to our company and to Missouri.
White-tailed deer and deer hunting are a multi-generational and important family heritage for many generations of Missouri families.
All white-tailed deer face serious threats from diseases such as Chronic Wasting Disease (CWD).
The Department has been working diligently to address disease management for white-tailed deer, including regulation changes to help slow the spread and limit the prevalence and impact of diseases such as CWD.
I know the Missouri Department of Conservation is currently taking the pulse of Missouri citizens on policy related to captive deer facilities and shooting preserves.
I also know that our citizens are supportive of taking steps to protect our deer population through improved regulations for deer breeding facilities and shooting preserves.
I am very much opposed to the override of the Governor’s veto.
An override could put our wild deer population in jeopardy.
We can’t take that chance.
Thank you for your consideration.
Letter of concern regarding CWD from Johnny Morris, Founder/CEO, Bass Pro Shops. (SEE URL LINK BELOW TO READ LETTER...TSS)
Impacts on wildlife economies
The evidence regarding the presence, growth, spread, persistence, evolution/adaptation, and impacts of CWD on wildlife are overwhelming. The full extent of the ecological harm will not be known for decades, but it will have direct impacts on human-wildlife interactions. While CWD impacts may vary across areas, ecosystems, species, and
people, the experiences and impacts documented in Wisconsin are worth noting.
“In early 2002, CWD was discovered in three wild white tailed deer in Wisconsin. Nine months later, hunting license sales had declined by over 90,000, revenue to the Wisconsin Department of Natural Resources (WDNR) had dropped by over $3,000,000 (Heberlein, 2004), and the economic loss was estimated at over $50,000,000 (Bishop, 2004).”217 To assess potential impacts against perceived risk, Vaske and Lyon “presented hunters with six scenarios depicting hypothetical CWD prevalence levels and human health risks from the disease (e.g., death), and asked if they would continue or stop hunting deer in the state.” Responses followed risk, culminating in 64% of respondents saying they would quit hunting under conditions of proven risk to people.218
Market-based threats
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Legal basis of perception over science
The substantial role of perception in both markets and public policy has been well documented in issues such as GMO food labeling.226 Specific to TSEs, the interaction of science, markets, perception, and policy was examined in law in Creekstone Premium Beef v. U.S. Department of Agriculture.227 After the finding of BSE in the U.S. in 2003, trade bans in the company’s key markets of Japan and Korea were costing Creekstone $200,000 per day. In order to protect their customers and restore access to valuable international markets, the company built a testing lab and trained their staff so they could test all their animals for BSE. The USDA rejected Creekstone’s request to
perform BSE testing, refused to sell them the test kits, and intervened to stop Creekstone from purchasing (the same) test kits internationally. In their June 4, 2004 letter USDA reasoned that “allowing a company to use a BSE test in a private marketing program is inconsistent with USDA’s mandate to ensure effective, scientifically sound testing for significant animal diseases and maintain domestic and international confidence in U.S. cattle and beef products.”228
Citing continued revenue losses even after the bans were lifted, to address persisting consumer fears about BSE, (i.e., customer perceptions about the safety of their products), the company demanded the right to test all their animals, and brought suit on March 23, 2006. On appeal the case centered on the USDA authority under the Virus-Serum-Toxin Act (VSTA), but of relevance here is that the substance, intent, and justification argued by both parties centered around the vital role, impact, and interaction of science, evidence, and perceptions when confronting uncertain risk.229 Whether or not consumers know the science, their fears are based on perceptions that Creekstone sought to address through more testing; they wanted only to increase, not replace USDA testing. Similarly, the issues argued by the USDA regarding potential false inferences because of the limitations of science, or inaccurate or invalid testing, and the questions of authority under VSTA are all, necessarily, questions of perceptions versus reality. Facts and perceptions are both relevant, as is their interaction in science, in policy and law, and in markets. All are at play and have been deemed applicable throughout vast areas of law, regulatory structures and protocol, and in international treaties and agreements. There is neither evidence nor a plausible theory to suggest this would exclude CWD.
As science and the UK Assessment points out, CWD is a proven threat to red deer, reindeer, sika deer, muntjac deer, European moose, and several species of circumpolar rodents. Merely raising the alarm about the threat of global transfer through CWD-contaminated plants or agricultural products, may catch the attention of foreign wildlife advocates as well as non-North American, highly motivated (multi $billion) competitive agricultural producers. This presents a substantial risk regarding the potential for trade restrictions on North American agriculture products.230 Should that happen, the effects of wildlife being condemned by agricultural interests as ‘infected vermin, shedding prions into landscapes and into agricultural lands and crops’ will be devastating. Even without media attention, the effect of prion contamination on property values has already been demonstrated as severe. The dozens of game farms cited earlier that remain under permanent CWD quarantine represent a greater than total loss of value, as fences have to be maintained to prevent disease transfers off the property. This will only increase as information regarding plant contamination becomes better known.
Unfortunately, our experience confirms that reactions to potential threats to agriculture from wildlife are real. Regardless of agriculture’s role or responsibility in causing the problem, wildlife will be routinely targeted. For example, elk and bison contracted bovine tuberculosis and bovine brucellosis from cattle.231 By the mid-20th century, concern over the possibility transmissions to cattle, and threats to valuable disease-free trade status, led to repeated culling of wildlife to protect agricultural interest. In Canada, even though bison lived in the far north Wood Buffalo National Park, and despite the value of the bison genome, the agriculture lobby demanded the complete elimination of bison to protect their interest.232
Consensus on urgency and vital actions
The preceding are but glimpses into the various disciplines, aspects, and complex factors involved in analyzing CWD, the existing impacts, and dynamic risks. It is abundantly clear, however, that the CWD crisis is, indeed “insidious and dire.” While details and methods must be guided by science and evidence, there is significant agreement on critical needs; and we have assurances from leading experts that we have the labs and capacity to meet this challenge.
Immediate action is required to avoid worst-case outcomes. We require mandate and funding to:
1. Contain the geographic spread of CWD by enacting and enforcing an immediate ban on the movement of all live cervids, all potentially CWD-infected carcasses, animal parts, products, exposed equipment, trailers, or other sources of infectious materials.
2. Mandate and implement for hunters, convenient, cost-free, rapid testing of all animals harvested from CWD-affected areas.
3. Ensure that no CWD-infected material reaches the food or feed chains, and that it is instead properly disposed of.
4. Establish and fund accountable research and science-based policy to protect public interest (health, wildlife and related industries, agriculture, our economies and communities).
It is important to note that consensus regarding these needs extends to optimism regarding the efficacy, the practical efficiency, and cost-effectiveness of the actions. Comprehensive analysis with vital stakeholder engagement will foster understanding, re-connection, and genuine appreciation of interrelated systems on which we depend. While dire, we have a unique opportunity to realize enormous advantages of cooperative effort and precautionary approach.
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FINALLY, SEE SINGELTARY ON NUTRITIONAL SUPPLEMENTS, PET FOOD, FEED, MISSOURI, CREEKSTONE, AND TSE PRION DISEASE...post at bottom...TSS
PRION 2017 CONFERENCE ABSTRACT First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1
University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen
This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.
Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.
At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS
TUESDAY, JUNE 13, 2017
PRION 2017 CONFERENCE ABSTRACT First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
Subject: CWD TRANSMITS TO MACAQUE ORALLY MUSCLE INTAKE
Notice to Members Regarding Chronic Wasting Disease (CWD)
Posted on: May 31st, 2017
To: MNA Members From: Métis Nation of Alberta
Date: Wednesday, May 31, 2017
Métis Nation of Alberta (MNA) was made aware of a recent Canadian research study examining the transmission of Chronic Wasting Disease. The initial results of the study indicate that macaque monkeys (genetically similar to humans) can be infected with Chronic Wasting Disease (CWD) after eating deer that is infected with CWD. CWD is a prion disease, which are fatal, transmissible diseases characterized by abnormal proteins in the brain and nervous system. To date no research has shown that CWD can be passed on to humans, and no human cases of CWD have ever been identified. However, this new research indicates that it is a possibility. The Deputy Chief Medical Officer of Health has reached out to us to share with our Métis harvesters this important information. For more information you can visit:
and
What the Alberta Government knows:
CWD is present in southeastern Alberta, with the area slowly spreading westward over time (introduced into Alberta from Saskatchewan) – see map for more information at
CWD circulates in deer populations, particularly mule deer; it has been found in about 4% of deer tested in 2016; Elk can be infected in areas where CWD has been present in deer for a long period of time; Moose can also be infected, but this would be fairly rare. Necessary Precautions for Harvesters: Hunters and others who handle carcasses follow basic handling precautions (available here
All deer, moose and elk harvested from CWD mandatory submission wildlife management units (WMUs) be tested for CWD; and A negative result (no CWD detected by the test) must be obtained before any part of an animal is eaten.
For more information, contact: Amy Quintal Métis Nation of Alberta Métis Harvesting Liaison Tel: (780) 455 – 2200 aquintal@metis.org
Chronic Wasting Disease: CFIA Research Summary
Embargoed until May 23, 2017
(OCR of a scanned original)
Research Findings
Chronic Wasting Disease (CWD) is a progressive, fatal disease of the nervous system of cervids including deer, elk, moose, and reindeer that is caused by abnormal proteins called prions. It is known as a transmissible spongiform encephalopathy (TSE). Other TSEs include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and Creutzfeldt-Jakob disease (CJD) in humans.
A limited number of experimental studies have demonstrated that non-human primates, specifically squirrel monkeys, are susceptible to CWD prions. An ongoing research study has now shown that CWD can also be transmitted to macaques, which are genetically closer to humans.
The study led by Dr. Stefanie Czub, a scientist at the Canadian Food Inspection Agency (CFIA), and funded by the Alberta Prion Research institute has demonstrated that by orally administering material under experimental conditions from cervids (deer and elk) naturally infected with CWD, the disease can be transmitted to macaques.
in this project, which began in 2009, 18 macaques were exposed to CWD in a variety of ways: by injecting into the brain, through contact with skin, oral administration, and intravenously (into the bloodstream through veins). So far, results are available from 5 animals. At this point, two animals that were exposed to CWD by direct introduction into the brain, one that was administered infected brain material by oral administration and two that were given infected muscle by oral administration have become infected with CWD. The study is ongoing and testing continues in the remaining animals. The early results will be presented at PRlON 2017, the annual international conference on prion diseases, in Edinburgh, Scotland, May 23 to 26, 2017.
Potential impacts of the new finding
Since 2003 Canada has a policy that recommends that animals and materials known to be infected with prions be removed from the food chain and from health products. Although no direct evidence of CWD prion transmission to humans has ever been recorded, the policy advocates a precautionary approach to managing CWD and potential human exposure to prions. These initial findings do not change Health Canada’s Health Products and Food Branch (HPFB) position on food and health products. A precautionary approach is still recommended to manage the potential risks of exposure to prions through food and health products. Measures are in place at federal, provincial and territorial levels to reduce human exposure to products potentially contaminated by CWD by preventing known infected animals from entering the marketplace.
While Federal and P/T government’s animal disease control policies continue to divert known CWD-infected animals away from entering the food and feed supply, research and development of sensitive detection methods including live-animal sampling techniques remain crucial for ensuring an accurate diagnosis. In addition, consistent federal, provincial and territorial communications of appropriate precautionary measures for hunters and indigenous communities are required.
Next Steps
The CFlA will continue to collaborate with national and international partners to develop and validate new diagnostic techniques. The CFlA will also continue to offer confirmatory testing services and reference laboratory expertise to provincial and territorial partners on demand.
Currently, CFlA laboratories are leading or collaborating on several research projects to understand the potential for CWD to infect humans. These projects use non‐human primates, genetically modified mice, and cell-free amplification approaches. Given the present findings, CFiA encourages continued research into TSEs.
The results of this study reinforce the need to redesign the federal program to foster greater adoption of risk mitigation measures for farmed cervids. Federal and provincial government collaboration will continue as new program options are assessed.
The results of Dr. Czub’s research into CWD will be of interest to scientists, governments, industry and people who consume cervid products. After the presentation at PRION 2017, the research will follow the normal steps of completion, peer review and publication. The Government of Canada will monitor the response to this research and determine whether further review of the science is required. Other studies underway by other researchers may also become public as a result of the presentation of Dr. Czub’s research.
The Public Health Agency of Canada, Health Canada, CFlA and other Federal partners are working together to assess what policies or programs need further review as well as preparing other communications about the research and health policy and advice to Canadian. 2017/04/28
===end...UNOFFICIAL...NO URL LINK...TSS===
0:30 First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
Dr Stefanie Czub University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency Canada
WEDNESDAY, MAY 03, 2017
*** First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques
seems if my primitive education does not fail me, intracranial means inside the skull, and peroral means by the mouth. seems the price of tse prion poker just keeps going up...terry
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Location: Virus and Prion Research
Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease
Author item Moore, Sarah item Kunkle, Robert item Kondru, Naveen item Manne, Sireesha item Smith, Jodi item Kanthasamy, Anumantha item West Greenlee, M item Greenlee, Justin
Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 3/15/2017 Publication Date: N/A Citation: N/A Interpretive Summary:
Technical Abstract: Aims: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of cervids. We previously demonstrated that disease-associated prion protein (PrPSc) can be detected in the brain and retina from pigs challenged intracranially or orally with the CWD agent. In that study, neurological signs consistent with prion disease were observed only in one pig: an intracranially challenged pig that was euthanized at 64 months post-challenge. The purpose of this study was to use an antigen-capture immunoassay (EIA) and real-time quaking-induced conversion (QuIC) to determine whether PrPSc is present in lymphoid tissues from pigs challenged with the CWD agent.
Methods: At two months of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 challenge="" groups="" month="" pigs="" remaining="" the="">6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). The retropharyngeal lymph node (RPLN) was screened for the presence of PrPSc by EIA and immunohistochemistry (IHC). The RPLN, palatine tonsil, and mesenteric lymph node (MLN) from 6-7 pigs per challenge group were also tested using EIA and QuIC.
Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 5="" 6="" at="" by="" detected="" eia.="" examined="" group="" in="" intracranial="" least="" lymphoid="" month="" months="" of="" one="" pigs="" positive="" prpsc="" quic="" the="" tissues="" was="">6 months group, 5/6 pigs in the oral <6 4="" and="" group="" months="" oral="">6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). Conclusions:
This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge.
CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease.
Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.
CONFIDENTIAL
EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY
While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...
we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.
Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....
snip...see much more here ;
WEDNESDAY, APRIL 05, 2017
Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease
TUESDAY, APRIL 18, 2017
*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP ***
Wednesday, May 24, 2017
PRION2017 CONFERENCE VIDEO UPDATE 23 – 26 May 2017 Edinburgh UPDATE 1
Subject: PRION2017 CONFERENCE VIDEO UPDATE 23 – 26 May 2017 Edinburgh
*see archives of previous Prion Conferences, the ones that are still available, scroll down towards bottom in this link.
FRIDAY, JUNE 02, 2017
Alberta Canada Chronic Wasting Disease (CWD) Surveillance Update: 2016/17 Final
MONDAY, MAY 29, 2017
Canada CCA optimistic over potential for revisions to OIE criteria for BSE negligible risk
WEDNESDAY, MAY 31, 2017
Texas New Exotic CWD Susceptible Species Rules Now in Effect
WEDNESDAY, MAY 17, 2017
CWD, TSE, PRION, Cattle, Pigs, Sheep, and Humans aka Mad Cow Disease
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys
http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true
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://collections.europarchive.org...einquiry.gov.uk/files/yb/1994/08/00004001.pdf
you can see more evidence here ;
http://chronic-wasting-disease.blogspot.com/2016/05/zoonotic-potential-of-cwd-prions-update.html
really, i would not care what anyone eats. if you want to eat cwd infected deer elk or any cervid, that would be your risk to take, should not bother me. BUT, when it starts to risk my family, my children, friends, that have some sort of medical procedure done, surgery, dental, tissue, blood, etc. then that exposure of cwd to humans by consumption goes on to risk other humans that may never have eaten cervid. now is the time to act folks, we have floundered and pass this down the line long enough now it's catching up, and it may be too late. the incubation period here is what is fooling everyone. same thing happened with scrapie, until now, we know scrapie is risk to humans, this is scientific facts, ignore them if you must, but know this, you are playing with fire folks...don't take my word on it. read the science, and then make you minds up if your gonna risk your loved ones with this. it's like playing russian roulette...
Research Article
Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.
- C J Gibbs, Jr,
- D M Asher,
- A Kobrine,
- H L Amyx,
- M P Sulima,
- D C Gajdusek
Author affiliations
Abstract
Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://dx.doi.org/10.1136/jnnp.57.6.757
0:30 First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
Dr Stefanie Czub University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency Canada
http://prion2017.org/programme/
''in this project, which began in 2009, 18 macaques were exposed to CWD in a variety of ways:
by injecting into the brain, through contact with skin, oral administration, and intravenously (into the bloodstream through veins).
So far, results are available from 5 animals.
At this point, two animals that were exposed to CWD by direct introduction into the brain,
one that was administered infected brain material by oral administration and
two that were given infected muscle by oral administration have become infected with CWD.
The study is ongoing and testing continues in the remaining animals. The early results will be presented at PRlON 2017, the annual international conference on prion diseases, in Edinburgh, Scotland, May 23 to 26, 2017.''
end...tss
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 reqarding 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
2009
7 Section Contents Menu
Recalls, Market Withdrawals, & Safety Alerts
8 Archive for Recalls, Market Withdrawals & Safety Alerts
9 2009
10 Recall -- Firm Press Release .
Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have Chronic Wasting Disease Contact: Exotic Meats USA 1-800-680-4375
FOR IMMEDIATE RELEASE -- February 9, 2009 -- Exotic Meats USA of San Antonio, TX is initiating a voluntary recall of Elk Tenderloin because it may contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD). The meat with production dates of December 29, 30 and 31, 2008 was purchased from Sierra Meat Company in Reno, NV.
The infected elk came from Elk Farm LLC in Pine Island, MN and was among animals slaughtered and processed at USDA facility Noah’s Ark Processors LLC. Chronic Wasting Disease (CWD) is a fatal brain and nervous system disease found in elk and deer.
The disease is caused by an abnormally shaped protein called a prion, which can damage the brain and nerves of animals in the deer family.
Currently, it is believed that the prion responsible for causing CWD in deer and elk is not capable of infecting humans who eat deer or elk contaminated with the prion, but the observation of animal-to-human transmission of other prion-mediated diseases, such as bovine spongiform encephalopathy (BSE), has raised a theoretical concern regarding the transmission of CWD from deer or elk to humans.
At the present time, FDA believes the risk of becoming ill from eating CWD-positive elk or deer meat is remote.
However, FDA strongly advises consumers to return the product to the place of purchase, rather than disposing of it themselves, due to environmental concerns.
Thursday, May 26, 2011
Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007
FoodNet Population Survey Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.
Elk meat recalled due to CWD Boulder County Health Department and Colorado Department of Public Health and Environment Warning out on elk meat sold at Boulder farmer's market
By Kevin Vaughan, Rocky Mountain News (Contact) Published December 25, 2008 at 12:05 a.m.
State health officials issued a warning Wednesday after learning that unsuspecting consumers bought hundreds of pounds of elk meat this month from an animal infected with chronic wasting disease.
The elk was sold Dec. 13 at a farmer's market at the Boulder County Fairgrounds.
Although research has found no risk to humans who eat infected elk, officials at the state and Boulder County health departments recommended that the meat not be consumed.
"There's been now 10 years- plus of research looking at whether CWD poses a human health risk, and the evidence to date suggests it does not," said John Pape, epidemiologist at the Colorado Department of Health and Environment.
Still, he said, the research is not definitive.
In all, 15 animals purchased at a commercial Colorado elk ranch were processed in early December at a USDA-licensed plant. All those animals were tested for the disease.
Test results obtained Tuesday indicated that one of the animals was infected with CWD, one of several diseases thought to be caused by misshaped proteins that inflict damage to nerve cells in the brain. It is a cousin to both crapie in sheep and mad cow disease.
Label information
On infected elk meat:
* Seller: High Wire Ranch
* Cuts: chuck roast, arm roast, flat iron, ribeye steak, New York steak, tenderloin, sirloin tip roast, medallions and ground meat.
* Processor: Cedaredge Processing
* USDA triangle number: 34645
For more information, contact John Pape, Colorado Department of Health and Environment, 303-692-2628.
Elk meat recalled due to wasting disease
Publish Date: 12/24/2008
Longmont Times-Call
LONGMONT — Meat from an elk with chronic wasting disease was sold Dec. 13 at the Boulder County Fairgrounds, according to the Colorado Department of Public Health and Environment. The meat is being recalled.
State health officials said the animal was one of 15 elk purchased from High Wire Ranch and processed in early December before being sold at a farmers market at the fairgrounds. The disease was found during a routine preliminary test for CWD; none of the other 14 elk were deemed to be infected.
CWD is not known to be dangerous to humans, health officials said, but the state advises against eating meat from animals with the disease.
The labeling on the meat would include:
• Seller: High Wire Ranch • The type of cut, listed as either “chuck roast,” “arm roast,” “flat iron,” “ribeye steak,” “New York steak,” “tenderloin,” “sirloin tip roast,” “medallions” or “ground meat.”
• Processor: Ceaderedge Processing • A USDA triangle with the number 34645. Final testing is still being conducted. State officials said the meat should be discarded if it matches the packaging label and was bought on the fairgrounds on Dec. 13.
CWD is a disease believed to be caused by prions, misshapen proteins that cause brain damage. The disease affects elk, deer and moose. Other prion diseases include scrapie in sheep and bovine spongiform encephalopathy or “mad cow disease” in cattle.
People with questions about the meat can contact John Pape of the state health department at 303-692-2628.
COLORADO: Farmer's market meat recalled after testing positive for CWD
24.dec.08 9News.com Jeffrey Wolf
Elk meat that was sold at a farmer's market is being recalled because tests show it was infected with chronic wasting disease. The Boulder County Health Department and Colorado Department of Public Health and Environment issued the recall Wednesday after the meat was sold at the Boulder County Fairgrounds on Dec. 13. Although there isn't any human health risk connected with CWD, the recalled was issued as a precaution. About 15 elk were bought from a commercial ranch in Colorado in early December and processed at a licensed plant. All 15 were tested for CWD and one came up positive. The labeling on the product would have the following information: *Seller: High Wire Ranch *The type of cut: "chuck roast," "arm roast," "flat iron," "ribeye steak," "New York steak," "tenderloin," "sirloin tip roast," "medallions" or "ground meat." *Processor: Cedaredge Processing *The USDA triangle containing the number "34645" People with questions about this meat can contact John Pape, epidemiologist at the Colorado Department of Public Health and Environment at 303-692-2628.
COULD NOT FIND any warning or recalls on these two sites confirming their recall of CWD infected meat. ...TSS
Envt.07:
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
Yet, it has to be noted that our assessments of PrPTSE levels in skeletal muscles were based on findings in presumably pre- or subclinically infected animals. Therefore, the concentration of PrPTSE in skeletal muscles of WTD with clinically manifest CWD may possibly exceed our estimate which refers to clinically inconspicuous animals that are more likely to enter the human food chain. Our tissue blot findings in skeletal muscles from CWD-infected WTD would be consistent with an anterograde spread of CWD prions via motor nerve fibres to muscle tissue (figure 4A). Similar neural spreading pathways of muscle infection were previously found in hamsters orally challenged with scrapie [28] and suggested by the detection of PrPTSE in muscle fibres and muscle-associated nerve fascicles of clinically-ill non-human primates challenged with BSE prions [29]. Whether the absence of detectable PrPTSE in myofibers observed in our study is a specific feature of CWD in WTD, or was due to a pre- or subclinical stage of infection in the examined animals, remains to be established. In any case, our observations support previous findings suggesting the precautionary prevention of muscle tissue from CWD-infected WTD in the human diet, and highlight the need to comprehensively elucidate of whether CWD may be transmissible to humans. While the understanding of TSEs in cervids has made substantial progress during the past few years, the assessment and management of risks possibly emanating from prions in skeletal muscles of CWD-infected cervids requires further research.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Rachel C. Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡, Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ + Author Affiliations
1 Department of Microbiology, Immunology and Molecular Genetics, University of Kentucky, Lexington, KY 40536, USA. 2 Sanders Brown Center on Aging, University of Kentucky, Lexington, KY 40536, USA. 3 Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4 Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA. ↵§ To whom correspondence should be addressed. E-mail:
gtell2@uky.edu ↵* These authors contributed equally to this work.
↵† Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, FL 33458, USA.
↵‡ Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.
Abstract The emergence of chronic wasting disease (CWD) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic potential of CWD. Because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.
Wednesday, April 06, 2011
Presence and Seeding Activity of Pathological Prion Protein (PrPTSE) in Skeletal Muscles of White-Tailed Deer Infected with Chronic Wasting Disease
Prion Infectivity in Fat of Deer with Chronic Wasting Disease
Brent Race,# Kimberly Meade-White,# Richard Race, and Bruce Chesebro* Rocky Mountain Laboratories, 903 South 4th Street, Hamilton, Montana 59840
Received 2 June 2009/ Accepted 24 June 2009
ABSTRACT Top ABSTRACT TEXT REFERENCES
Chronic wasting disease (CWD) is a neurodegenerative prion disease of cervids. Some animal prion diseases, such as bovine spongiform encephalopathy, can infect humans; however, human susceptibility to CWD is unknown. In ruminants, prion infectivity is found in central nervous system and lymphoid tissues, with smaller amounts in intestine and muscle. In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.
snip...
The highest risk of human contact with CWD might be through exposure to high-titer CNS tissue through accidental skin cuts or corneal contact at the time of harvest and butchering. However, the likelihood of a human consuming fat infected with a low titer of the CWD agent is much higher. It is impossible to remove all the fat present within muscle tissue, and fat consumption is inevitable when eating meat. Of additional concern is the fact that meat from an individual deer harvested by a hunter is typically consumed over multiple meals by the same group of people. These individuals would thus have multiple exposures to the CWD agent over time, which might increase the chance for transfer of infection.
In the Rocky Mountain region of North America, wild deer are subject to predation by wolves, coyotes, bears, and mountain lions. Although canines such as wolves and coyotes are not known to be susceptible to prion diseases, felines definitely are susceptible to BSE (9) and might also be infected by the CWD agent. Deer infected with the CWD agent are more likely to be killed by predators such as mountain lions (11). Peripheral tissues, including lymph nodes, muscle, and fat, which harbor prion infectivity are more accessible for consumption than CNS tissue, which has the highest level of infectivity late in disease. Therefore, infectivity in these peripheral tissues may be important in potential cross-species CWD transmissions in the wild.
The present finding of CWD infectivity in deer fat tissue raises the possibility that prion infectivity might also be found in fat tissue of other infected ruminants, such as sheep and cattle, whose fat and muscle tissues are more widely distributed in both the human and domestic-animal food chains. Although the infectivity in fat tissues is low compared to that in the CNS, there may be significant differences among species and between prion strains. Two fat samples from BSE agent-infected cattle were reported to be negative by bioassay in nontransgenic RIII mice (3, 6). However, RIII mice are 10,000-fold-less sensitive to BSE agent infection than transgenic mice expressing bovine PrP (4). It would be prudent to carry out additional infectivity assays on fat from BSE agent-infected cattle and scrapie agent-infected sheep using appropriate transgenic mice or homologous species to determine the risk from these sources.
0C7.04
North American Cervids Harbor Two Distinct CWD Strains
Authors
Angers, R. Seward, T, Napier, D., Browning, S., Miller, M., Balachandran A., McKenzie, D., Hoover, E., Telling, G. 'University of Kentucky; Colorado Division of Wildlife, Canadian Food Inspection Agency; University Of Wisconsin; Colorado State University.
Content
Despite the increasing geographic distribution and host range of CWD, little is known about the prion strain(s) responsible for distinct outbreaks of the disease. To address this we inoculated CWD-susceptible Tg(CerPrP)1536+/· mice with 29 individual prion samples from various geographic locations in North America. Upon serial passage, intrastudy incubation periods consistently diverged and clustered into two main groups with means around 210 and 290 days, with corresponding differences in neuropathology. Prion strain designations were utilized to distinguish between the two groups: Type I CWD mice succumbed to disease in the 200 day range and displayed a symmetrical pattern of vacuolation and PrPSc deposition, whereas Type II CWD mice succumbed to disease near 300 days and displayed a strikingly different pattern characterized by large local accumulations of florid plaques distributed asymmetrically. Type II CWD bears a striking resemblance to unstable parental scrapie strains such as 87A which give rise to stable, short incubation period strains such as ME7 under certain passage conditions. In agreement, the only groups of CWD-inoculated mice with unwavering incubation periods were those with Type I CWD. Additionally, following endpoint titration of a CWD sample, Type I CWD could be recovered only at the lowest dilution tested (10-1), whereas Type II CWD was detected in mice inoculated with all dilutions resulting in disease. Although strain properties are believed to be encoded in the tertiary structure of the infectious prion protein, we found no biochemical differences between Type I and Type II CWD. Our data confirm the co·existence of two distinct prion strains in CWD-infected cervids and suggest that Type II CWD is the parent strain of Type I CWD.
see page 29, and see other CWD studies ;
Sunday, November 23, 2008
PRION October 8th - 10th 2008 Book of Abstracts
ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A WISCONSIN STRAIN OF CWD
Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5
The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region.
now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From: Sent: Sunday, September 29, 2002 10:15 AM
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
*** WDA 2016 NEW YORK ***
We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions.
In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species.
We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions.
Student Presentations Session 2
The species barriers and public health threat of CWD and BSE prions
Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr. Edward Hoover1 1Colorado State University
Chronic wasting disease (CWD) is spreading rapidly through cervid populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease) arose in the 1980s because cattle were fed recycled animal protein. These and other prion diseases are caused by abnormal folding of the normal prion protein (PrP) into a disease causing form (PrPd), which is pathogenic to nervous system cells and can cause subsequent PrP to misfold. CWD spreads among cervids very efficiently, but it has not yet infected humans. On the other hand, BSE was spread only when cattle consumed infected bovine or ovine tissue, but did infect humans and other species. The objective of this research is to understand the role of PrP structure in cross-species infection by CWD and BSE. To study the propensity of each species’ PrP to be induced to misfold by the presence of PrPd from verious species, we have used an in vitro system that permits detection of PrPd in real-time. We measured the conversion efficiency of various combinations of PrPd seeds and PrP substrate combinations. We observed the cross-species behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species.
***We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions.
CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD.
Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders
PRION 2016 TOKYO
Zoonotic Potential of CWD Prions: An Update
Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions.
PRION 2016 TOKYO In Conjunction with Asia Pacific Prion Symposium 2016 PRION 2016 Tokyo Prion 2016
Cervid to human prion transmission
Kong, Qingzhong
Case Western Reserve University, Cleveland, OH, United States
Abstract
Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;
(3) Reliable essays can be established to detect CWD infection in humans; and
(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.
Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3.
Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1.
Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.
Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans.
Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans.
Key Molecular Mechanisms of TSEs
Zabel, Mark D.
Colorado State University-Fort Collins, Fort Collins, CO, United States
Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative diseases affecting humans, cervids, bovids, and ovids. The absolute requirement of PrPC expression to generate prion diseases and the lack of instructional nucleic acid define prions as unique infectious agents. Prions exhibit species-specific tropism, inferring that unique prion strains exist that preferentially infct certain host species and confront transmission barriers to heterologous host species. However, transmission barriers are not absolute. Scientific consensus agrees that the sheep TSE scrapie probably breached the transmission barrier to cattle causing bovine spongiform encephalopathy that subsequently breached the human transmission barrier and likely caused several hundred deaths by a new-variant form of the human TSE Creutzfeldt-Jakob disease in the UK and Europe. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease of cervids, overcoming similar human transmission barriers. A groundbreaking discovery made last year revealed that mice infected with heterologous prion strains facing significant transmission barriers replicated prions far more readily in spleens than brains6. Furthermore, these splenic prions exhibited weakened transmission barriers and expanded host ranges compared to neurogenic prions. These data question conventional wisdom of avoiding neural tissue to avoid prion xenotransmission, when more promiscuous prions may lurk in extraneural tissues. Data derived from work previously funded by NIH demonstrate that Complement receptors CD21/35 bind prions and high density PrPC and differentially impact prion disease depending on the prion isolate or strain used. Recent advances in live animal and whole organ imaging have led us to generate preliminary data to support novel, innovative approaches to assessing prion capture and transport. We plan to test our unifying hypothesis for this proposal that CD21/35 control the processes of peripheral prion capture, transport, strain selection and xenotransmission in the following specific aims.
1. Assess the role of CD21/35 in splenic prion strain selection and host range expansion.
2. Determine whether CD21/35 and C1q differentially bind distinct prion strains
3. Monitor the effects of CD21/35 on prion trafficking in real time and space
4. Assess the role of CD21/35 in incunabular prion trafficking
Public Health Relevance Transmissible spongiform encephalopathies, or prion diseases, are devastating illnesses that greatly impact public health, agriculture and wildlife in North America and around the world. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease (CWD) of cervids, overcoming similar human transmission barriers. Early this year Canada reported its first case of BSE in over a decade audits first case of CWD in farmed elk in three years, underscoring the need for continued vigilance and research. Identifying mechanisms of transmission and zoonoses remains an extremely important and intense area of research that will benefit human and other animal populations.
PMCA Detection of CWD Infection in Cervid and Non-Cervid Species
Hoover, Edward Arthur
Colorado State University-Fort Collins, Fort Collins, CO, United States
LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
Molecular Barriers to Zoonotic Transmission of Prions
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
SATURDAY, MAY 20, 2017
Missouri CWD TSE PRION Surveillance and Monitoring
THURSDAY, MARCH 09, 2017
Missouri MDC REPORTS TWO CASES OF CWD IN ST. CLAIR COUNTY
Tuesday, August 16, 2016
Docket No. FDA-2011-D-0376 Dietary Supplements: New Dietary Ingredient Notifications and Related Issues; Revised Draft Guidance for Industry Singeltary Submission
SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY
***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.***
Saturday, April 23, 2016
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
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a, Natalia Fernandez-Borges a. and Alba Marin-Moreno a "Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier. To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents. These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant. 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.
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.
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
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.
LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, ***
*** and there may be asymptomatic individuals infected with the CWD equivalent.
*** These circumstances represent a potential threat to blood, blood products, and plasma supplies.
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
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...
R. BRADLEY
Prion, 11:136–142, 2017 Published with license by Taylor & Francis ISSN: 1933-68961933-690X online DOI: 10.1080/19336896.2017.1300741
High prevalence of prion protein genotype associated with resistance to chronic wasting disease in one Alberta woodland caribou population
Yo Ching Chenga , Marco Musianib , Maria Cavedonb , and Sabine Gilcha a Department of Ecosystem and Public Health, Calgary Prion Research Unit, Faculty of Veterinary Medicine, University of Calgary, Calgary, Canada; b Department of Biological Sciences, Faculty of Science, University of Calgary, Calgary, Canada ABSTRACT.
Chronic wasting disease (CWD) is a prion disease found in deer, elk and moose in North America and since recently, wild reindeer in Norway. Caribou are at-risk to encounter CWD in areas such as Alberta, Canada, where the disease spreads toward caribou habitats. CWD susceptibility is modulated by species-specific polymorphisms in the prion protein gene (Prnp). We sequenced Prnp of woodland caribou from 9 Albertan populations. In one population (Chinchaga) a significantly higher frequency of the 138N allele linked to reduced CWD susceptibility was observed. These data are relevant for developing CWD management strategies including conservation of threatened caribou populations.
KEYWORDS. caribou, chronic wasting disease, conservation, genetic resistance, prion protein, prion protein gene polymorphism
***at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified.
P-145 Estimating chronic wasting disease resistance in cervids using real time quaking- induced conversion
Nicholas J Haley1, Rachel Rielinqer2, Kristen A Davenport3, W. David Walter4, Katherine I O'Rourke5, Gordon Mitchell6, Juergen A Richt2
1 Department of Microbiology and Immunology, Midwestern University, United States; 2Department of Diagnostic Medicine and Pathobiology, Kansas State University; 3Prion Research Center; Colorado State University; 4U.S. Geological Survey, Pennsylvania Cooperative Fish and Wildlife Research Unit; 5Agricultural Research Service, United States Department of Agriculture; 6Canadian Food Inspection Agency, National and OlE Reference Laboratory for Scrapie and CWO
In mammalian species, the susceptibility to prion diseases is affected, in part, by the sequence of the host's prion protein (PrP). In sheep, a gradation from scrapie susceptible to resistant has been established both in vivo and in vitro based on the amino acids present at PrP positions 136, 154, and 171, which has led to global breeding programs to reduce the prevalence of scrapie in domestic sheep. In cervids, resistance is commonly characterized as a delayed progression of chronic wasting disease (CWD); at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified. To model the susceptibility of various naturally-occurring and hypothetical cervid PrP alleles in vitro, we compared the amplification rates and efficiency of various CWD isolates in recombinant PrPC using real time quaking-induced conversion. We hypothesized that amplification metrics of these isolates in cervid PrP substrates would correlate to in vivo susceptibility - allowing susceptibility prediction for alleles found at 10 frequency in nature, and that there would be an additive effect of multiple resistant codons in hypothetical alleles. Our studies demonstrate that in vitro amplification metrics predict in vivo susceptibility, and that alleles with multiple codons, each influencing resistance independently, do not necessarily contribute additively to resistance. Importantly, we found that the white-tailed deer 226K substrate exhibited the slowest amplification rate among those evaluated, suggesting that further investigation of this allele and its resistance in vivo are warranted to determine if absolute resistance to CWD is possible.
***at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified.
PRION 2016 CONFERENCE TOKYO
CWD of deer and elk is spreading across North America and cannot be stopped.
The tse prion aka mad cow type disease is not your normal pathogen.
The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.
You cannot cook the TSE prion disease out of meat. you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.
The TSE prion agent also survives Simulated Wastewater Treatment Processes.
IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.
You can bury it and it will not go away.
The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.
it’s not your ordinary pathogen you can just cook it out and be done with.
that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.
cwd to humans, consumption, exposure, sub-clinical, iatrogenic, what if ?
i strenuously urge you all to rethink this cutting of funds for research of the TSE Prion disease.
*** DECEMBER 2016 CDC EMERGING INFECTIOUS DISEASE JOURNAL CWD HORIZONTAL TRANSMISSION
*** INFECTIOUS AGENT OF SHEEP SCRAPIE MAY PERSIST IN THE ENVIRONMENT FOR AT LEAST 16 YEARS ***
GUDMUNDUR GEORGSSON1, SIGURDUR SIGURDARSON2 AND PAUL BROWN3
Title: Pathological features of chronic wasting disease in reindeer and demonstration of horizontal transmission
Using in vitro prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.
Claudio Soto Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.
***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.
========================
Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.
In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.
Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***
*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
with CWD TSE Prions, I am not sure there is any absolute yet, other than what we know with transmission studies, and we know tse prion kill, and tse prion are bad. science shows to date, that indeed soil, dirt, some better than others, can act as a carrier. same with objects, farm furniture. take it with how ever many grains of salt you wish, or not. if load factor plays a role in the end formula, then everything should be on the table, in my opinion...tss
Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil Particles
Author Summary
Transmissible spongiform encephalopathies (TSEs) are a group of incurable neurological diseases likely caused by a misfolded form of the prion protein. TSEs include scrapie in sheep, bovine spongiform encephalopathy (‘‘mad cow’’ disease) in cattle, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Scrapie and chronic wasting disease are unique among TSEs because they can be transmitted between animals, and the disease agents appear to persist in environments previously inhabited by infected animals. Soil has been hypothesized to act as a reservoir of infectivity and to bind the infectious agent. In the current study, we orally dosed experimental animals with a common clay mineral, montmorillonite, or whole soils laden with infectious prions, and compared the transmissibility to unbound agent. We found that prions bound to montmorillonite and whole soils remained orally infectious, and, in most cases, increased the oral transmission of disease compared to the unbound agent. The results presented in this study suggest that soil may contribute to environmental spread of TSEs by increasing the transmissibility of small amounts of infectious agent in the environment.
tse prion soil
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.
>>>Particle-associated PrPTSE molecules may migrate from locations of deposition via transport processes affecting soil particles, including entrainment in and movement with air and overland flow. <<<
Fate of Prions in Soil: A Review
Christen B. Smith, Clarissa J. Booth, and Joel A. Pedersen*
Several reports have shown that prions can persist in soil for several years. Significant interest remains in developing methods that could be applied to degrade PrPTSE in naturally contaminated soils. Preliminary research suggests that serine proteases and the microbial consortia in stimulated soils and compost may partially degrade PrPTSE. Transition metal oxides in soil (viz. manganese oxide) may also mediate prion inactivation. Overall, the effect of prion attachment to soil particles on its persistence in the environment is not well understood, and additional study is needed to determine its implications on the environmental transmission of scrapie and CWD.
P.161: Prion soil binding may explain efficient horizontal CWD transmission
Conclusion. Silty clay loam exhibits highly efficient prion binding, inferring a durable environmental reservoir, and an efficient mechanism for indirect horizontal CWD transmission.
>>>Another alternative would be an absolute prohibition on the movement of deer within the state for any purpose. While this alternative would significantly reduce the potential spread of CWD, it would also have the simultaneous effect of preventing landowners and land managers from implementing popular management strategies involving the movement of deer, and would deprive deer breeders of the ability to engage in the business of buying and selling breeder deer. Therefore, this alternative was rejected because the department determined that it placed an avoidable burden on the regulated community.<<<
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission
Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1
1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK
Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.
snip...
Discussion
Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).
Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing. Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building. Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9). The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture. When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.
This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep. Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease. It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled. Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases. Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.
PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice. In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals. In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay. False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28). This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm. This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc. In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc. Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material. In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12). A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30). This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model. Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.
In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.
Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***
*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
MONDAY, JUNE 05, 2017
Arkansas CWD Management Zone expands to include Van Buren County 213 cases to date
SUNDAY, MAY 21, 2017
Arkansas Chronic Wasting Disease CWD TSE Prion Roundup 212 Cases Confirmed To Date
THURSDAY, MAY 18, 2017
Minnesota Four more farmed white-tailed deer test positive for Chronic Wasting Disease CWD TSE Prion
MONDAY, MARCH 27, 2017
Wyoming CWD Postive Mule Deer Doe Near Pinedale
MONDAY, MARCH 20, 2017
Wisconsin CWD TSE Prion Annual Roundup 441 positive
TUESDAY, MARCH 14, 2017
Iowa 12 deer test positive for chronic wasting disease from 2016-17 hunting seasons
MONDAY, MARCH 13, 2017
CHRONIC WASTING DISEASE CWD TSE PRION UDATE March 13, 2017
FRIDAY, MARCH 10, 2017
Nebraska Tests confirm spread of CWD to Lancaster County
SATURDAY, MAY 20, 2017
Missouri CWD TSE PRION Surveillance and Monitoring
THURSDAY, MARCH 09, 2017
Missouri MDC REPORTS TWO CASES OF CWD IN ST. CLAIR COUNTY
SATURDAY, MARCH 04, 2017
Maryland DNR Six Deer Test Positive for Chronic Wasting Disease
WEDNESDAY, MARCH 01, 2017
South central Pennsylvania Captive Deer Tests Positive for Chronic Wasting Disease
MONDAY, MAY 15, 2017
Pennsylvania 25 more deer test positive for CWD TSE PRION in the wild
WEDNESDAY, MAY 31, 2017
Texas New Exotic CWD Susceptible Species Rules Now in Effect
MONDAY, MAY 15, 2017
TEXAS New CWD TSE PRION Case Discovered at Fifth Captive Deer Breeding Facility
SUNDAY, MAY 14, 2017
85th Legislative Session 2017 AND THE TEXAS TWO STEP Chronic Wasting Disease CWD TSE Prion, and paying to play
FRIDAY, MARCH 31, 2017
TPWD UPDATE CWD TSE Prion 49 confirmed cases and unwanted firsts for Texas
MONDAY, MARCH 13, 2017
CHRONIC WASTING DISEASE CWD TSE PRION UDATE March 13, 2017
SATURDAY, JANUARY 14, 2017
CHRONIC WASTING DISEASE CWD TSE PRION GLOBAL UPDATE JANUARY 14, 2017
WEDNESDAY, MAY 17, 2017
CWD, TSE, PRION, Cattle, Pigs, Sheep, and Humans aka Mad Cow Disease
MONDAY, APRIL 17, 2017
Wildlife advocates see wolves as 'best natural defense' against chronic wasting disease
NO WAY! this is an extremely stupid move, and very, very, dangerous...
MONDAY, MARCH 8, 2010
Canine Spongiform Encephalopathy aka MAD DOG DISEASE
TUESDAY, APRIL 18, 2017
*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP ***
WEDNESDAY, MAY 17, 2017
SHIC FUNDED STUDY SUGGESTS POTENTIAL FOR PATHOGEN TRANSMISSION VIA FEED
WEDNESDAY, MAY 17, 2017
CWD, TSE, PRION, Cattle, Pigs, Sheep, and Humans aka Mad Cow Disease
WEDNESDAY, MAY 17, 2017
*** Chronic Wasting Disease CWD TSE Prion aka Mad Deer Disease and the Real Estate Market Land Values ***
THURSDAY, MARCH 30, 2017
Norway CWD Skrantesjuke: VKM report supports the National Veterinary Institute perception management
Terry S. Singeltary Sr. Declares a DECLARATION OF EXTRAORDINARY EMERGENCY DUE TO A FOREIGN ANIMAL DISEASE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION CHRONIC WASTING DISEASE CWD IN THE UNITED STATES AND NORTH AMERICA.
The elephant in the room I was speaking of that we all have missed was the feed, yes we all know of ruminant and non ruminant protein and risk factors there from with TSE Prion disease, but we missed the rest of the feed i.e. FEED GRAINS. YES, science has shown in the past, and now recently, the shedding of the CWD TSE Prion into the environment is indeed a risk factor, and for all the grains and such that goes into feed, even hay, hell, Norway does not require any APHIS-Veterinary Services certification for the import of hay/straw. see for yourself ;
Hay/Straw
*** Norway does not require any APHIS-Veterinary Services certification for the import of hay/straw. ***
you add up all the other grains in feed, and then wonder about exposure to the CWD TSE PRION from cervid and risk factor from the CWD there from via shedding or right down to the soil these grains were grown in, and you have a world of problems.
see ;
Feed Grains Data: Yearbook Tables Created March 10, 2016
Updates of this data, and data covering more years and countries, can be found at
U.S. Acreage, Production, Yield, and Farm Price Table 1--Corn, sorghum, barley, and oats: Planted acreage, harvested acreage, production, yield, and farm price World Production, Supply, and Disappearance
Table 2--Foreign coarse grains: Supply and disappearance Table 3--Feed grains (corn, sorghum, barley, and oats): Supply and disappearance U.S. Supply and Disappearance Table 4--Corn: Supply and disappearance Table 5--Sorghum: Supply and disappearance Table 6--Barley: Supply and disappearance Table 7--Oats: Supply and disappearance U.S. Production, Yield, and Stocks Table 8--Hay: Production, harvested acreage, yield, and stocks Domestic and International Prices Table 9--Corn and sorghum: Average prices received by farmers, United States Table 10--Barley and oats: Average prices received by farmers, United States Table 11--Hay: Average prices received by farmers, United States Table 12--Corn: Cash prices at principal markets Table 13--Sorghum: Cash prices at principal markets Table 14--Barley and oats: Cash prices at principal markets Table 15--Feed-price ratios for livestock, poultry, and milk Table 16--Byproduct feeds: Average wholesale price, bulk, specified markets Table 17--Processed corn products: Quoted market prices Exports and Imports Table 18--U.S. corn and sorghum exports Table 19--U.S. barley and oats exports Table 20--U.S. corn and sorghum imports Table 21--U.S. barley and oats imports Table 22--U.S. corn and sorghum exports by selected destinations Table 23--U.S. barley and oats exports by selected destinations Table 24--U.S. corn and sorghum imports by selected sources Table 25--U.S. barley and oats imports by selected sources Table 26--U.S. white corn exports by selected destinations Table 27--World coarse grain trade: Selected exporters and importers by commodity Rail rates and shipments Table 28--Rail rates and grain shipments Processed feeds and animal unit indexes Table 29--Processed feeds: Quantities fed and feed per grain-consuming animal unit Table 30--Indexes of feed consuming animal units Feed, seed, and industrial uses Table 31—Corn: Feed, seed, and industrial uses Exports and imports for ethyl alcohol and brewers’ and distillers’ dregs and waste Table 32—U.S. exports of ethyl alcohol by selected destinations Table 33—U.S. imports of ethyl alcohol by selected sources Table 34—U.S. exports of brewers’ and distillers’ dregs and waste by selected commodities Table 35—U.S. imports of brewers’ and distillers’ dregs and waste by selected sources Contact: Thomas Capehart at tcapehart+A25@ers.usda.gov
‘’The statement you were concerned about was corrected to "One sorghum DDGS out of 168 DG samples was contaminated with animal protein prohibited for use in ruminant feed and was channeled to poultry feed."
Subject: Re: TEXAS CONFIRMATION OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION IN ONE SAMPLE OF SORGHUM DDGS OUT OF 168 DG SAMPLES
snip...see full text ;
PET FOOD, FSE AND CSE TSE PRION
YOU explained that imported crushed heads were extensively used in the petfood industry...
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
on occassions, materials obtained from slaughterhouses will be derived from sheep affected with scrapie or cattle that may be incubating BSE for use in petfood manufacture...
*** Meldrum's notes on pet foods and materials used
*** BSE & Pedigree Petfoods ***
SEE MUCH MORE HERE;
Tuesday, June 11, 2013
Weld County Bi-Products dba Fort Morgan Pet Foods 6/1/12 significant deviations from requirements in FDA regulations that are intended to reduce the risk of bovine spongiform encephalopathy (BSE) within the United States
THURSDAY, APRIL 07, 2016
What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016
Saturday, December 01, 2007
Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model
Sunday, December 10, 2006
Transmissible Mink Encephalopathy TME
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
Wednesday, April 25, 2012
4th MAD COW DISEASE U.S.A. CALIFORNIA ATYPICAL L-TYPE BSE 2012
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan.
*** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these countries. ***
see page 176 of 201 pages...tss
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
Wednesday, July 15, 2015
Additional BSE TSE prion testing detects pathologic lesion in unusual brain location and PrPsc by PMCA only, how many cases have we missed?
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.
*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***
Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT
MONDAY, JANUARY 16, 2017
APHIS Bovine Spongiform Encephalopathy (BSE): Ongoing Surveillance Program Last Modified: Jan 5, 2017
ONE DECADE POST MAD COW FEED BAN OF AUGUST 1997...
2007
10,000,000 POUNDS 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.
2007 Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II PRODUCT
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007 CODE Cattle feed delivered between 01/12/2007 and 01/26/2007 RECALLING FIRM/MANUFACTURER Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
REASON Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI
___________________________________
PRODUCT Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007
CODE The firm does not utilize a code - only shipping documentation with commodity and weights identified.
RECALLING FIRM/MANUFACTURER Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007.
Firm initiated recall is complete.
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.
VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs.
DISTRIBUTION ID and NV END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
NEW URL LINK;
another NEWER LINK;
TUESDAY, JANUARY 17, 2017
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE 2016 to 2017 BSE TSE PRION
FRIDAY, AUGUST 29, 2008
CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW
SUNDAY, APRIL 16, 2017
MM1-type sporadic Creutzfeldt-Jakob disease with 1-month total disease duration and early pathologic indicators
WEDNESDAY, APRIL 12, 2017
Case-control study on the use of pituitary-derived hormones from sheep as a potential risk factor for the occurrence of atypical scrapie in Great Britain
TUESDAY, SEPTEMBER 13, 2016
Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients
MONDAY, APRIL 03, 2017
Accessing transmissibility and diagnostic marker of skin prions
SUNDAY, DECEMBER 04, 2016
Heidenhain variant of Creutzfeldt–Jakob disease in a patient who had bovine bioprosthetic valve implantation
SUNDAY, JANUARY 17, 2016
Of Grave Concern Heidenhain Variant Creutzfeldt Jakob Disease
TUESDAY, APRIL 04, 2017
Please Support Funding for CDC and NPDPSC's Prion Disease Programs
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original Xavier Bosch
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt—Jakob ...
RE: re-Human Prion Diseases in the United States part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT No competing interests declared.
see full text ;
*** Needless conflict ***
Nature 485, 279–280 (17 May 2012) doi:10.1038/485279b
Published online 16 May 2012
Terry S. Singeltary Sr. said:
I kindly wish to submit the following please ;
Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
Monday, January 08, 2001 3:03 PM
CJD/BSE (aka madcow) Human/Animal TSE’s--U.S.--Submission To Scientific Advisors and Consultants Staff January 2001 Meeting (short version)
Greetings again Dr. Freas and Committee Members,
I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee...
snip... I think you are all aware of CJD vs vCJD, but i don't think you all know the facts of human/animal TSE's as a whole, they are all very very similar, and are all tied to the same thing, GREED and MAN.
I am beginning to think that the endless attempt to track down and ban, potential victims from known BSE Countries from giving blood will be futile. You would have to ban everyone on the Globe eventually? AS well, I think we MUST ACT SWIFTLY to find blood test for TSE's, whether it be blood test, urine test, eyelid test, anything at whatever cost, we need a test FAST.
DO NOT let the incubation time period of these TSEs fool you. To think of Scrapie as the prime agent to compare CJD, but yet overlook the Louping-ill vaccine event in 1930's of which 1000's of sheep where infected by scrapie from a vaccine made of scrapie infected sheep brains, would be foolish. I acquired this full text version of the event which was recorded in the Annual Congress of 1946 National Vet. Med. Ass. of Great Britain and Ireland.
From the BVA and the URL is posted in my (long version).
U.S.A. should make all human/animal TSE's notifiable at all ages, with requirements for a thorough surveillance and post-mortem examinations free of charge, if you are serious about eradicating this horrible disease in man and animal. ...
snip...see full text ;
THURSDAY, MARCH 30, 2017
Amyloid‑β accumulation in the CNS in human growth hormone recipients in the UK
SUNDAY, AUGUST 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
TUESDAY, AUGUST 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
CBCnews
*** USA sporadic CJD MAD COW DISEASE HAS HUGE PROBLEM Video
*** sporadic CJD linked to mad cow disease
*** you can see video here and interview with Jeff's Mom, and scientist telling you to test everything and potential risk factors for humans **
1994-10-13: Scrapie Man
*** Scrapie Video
1997-11-10: Panorama - The british disease
*** Human Mad Cow Video
2009-08-27
PrioNet Canada_Lecture "New Findings in Prion Research"
Prof. Dr. Adriano Aguzzi
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis *video*
Groups seek to save NIH brain collection
By STEVE MITCHELL, Medical Correspondent | April 1, 2005 at 4:48 PM
NIH may destroy human brain collection
By STEVE MITCHELL, Medical Correspondent | March 24, 2005 at 8:42 AM
NIH sends mixed signals on CJD brains
By STEVE MITCHELL, Medical Correspondent | April 7, 2005 at 3:30 PM
Groups seek to save NIH brain collection
By STEVE MITCHELL, Medical Correspondent | April 1, 2005 at 4:48 PM
JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005
Mr. Terry Singeltary P.O. Box 42 Bacliff, Texas 77518
Dear Mr. Singeltary:
In response to your recent request for my assistance, I have contacted the National Institutes of Health. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be of service in this matter.
Sincerely, JOHN CORNYN United States Senator JC:djl
===============
JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 May 18,2005 Mr. Terry Singeltary P.O. Box 42 Bacliff, Texas 77518
Dear Mr. Singeltary:
Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me.
Sincerely, JOHN CORNYN United States Senate JC:djl Enclosure
DEPARTMENT OF HEALTH & HUMAN SERVICES
National Institutes of Health National Institute of Neurological Disorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: [log in to unmask] May 10, 2005
The Honorable John Cornyn United States Senator Occidental Tower 5005 LBJ Freeway, Suite 1150 Dallas, Texas 75244-6199
Dear Senator Cornyn:
Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply.
Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.
I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved.
I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that a red is carded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification.)
The purpose of gathering these brains and tissues is to help scientists learn about CJD.
To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate that they have a compelling research or public health need for such materials.
For example, samples have been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National Prion Diseases Pathology Surveillance Center at Case Western Reserve University in Ohio and works with the Centers for Disease Control and Prevention to monitor all cases of CJD in the United States.
Dr. Gambetti studies the tissues to learn about the formation, physical and chemical properties, and pathogenic mechanisms of prion proteins, which are believed to be involved in the cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food and Drug Administration, for use in assessing a potential diagnostic test for CJD.
Page 2 - The Honorable John Cornyn
in closing, we know that donating organs and tissue from loved ones is a very difficult and personal choice that must often be made at the most stressful of times. We at the NINDS are grateful to those stalwart family members who make this choice in the selfless hope that it will help others afflicted with CJD. We also know the invaluable contribution such donations make to the advancement of medical science, and we are dedicated to the preservation of all of the tissue samples that can help in our efforts to overcome CJD.
I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely, Story C. Landis, Ph.D. Director, National Institute of Neurological Disorders and Stroke
==================================
CHRONIC WASTING DISEASE CWD TSE PRION AKA MAD DEER DISEASE AND FRIENDLY FIRE, PASS IT FORWARD, IATROGENIC MODE OF TRANSMISSION, WHAT IF?
Research Article
Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.
- C J Gibbs, Jr,
- D M Asher,
- A Kobrine,
- H L Amyx,
- M P Sulima,
- D C Gajdusek
Author affiliations
Abstract
Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://dx.doi.org/10.1136/jnnp.57.6.757
Wednesday, May 24, 2017
PRION2017 CONFERENCE VIDEO UPDATE 23 – 26 May 2017 Edinburgh UPDATE 1
In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells
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...see full text ;
Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells 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. ...
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 veiwed it as a wildlife problem and consequently not their province! ...page 26.
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
Terry S. Singeltary Sr.
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