CWD and Deer Diseases

Species Barrier May Protect Macaques from Chronic Wasting Disease

flounder — Tue, 04 Aug 2009 09:51:17 GMT

Greetings BuckMasters !

The following study is good news, but do NOT let this study fool anyone. It proves nothing for humans. well, at least that is what the officials have been telling us for decades when they transmit all these other TSEs to all these other species, ''oh, that they DID transmit to mouse, to mole, to hamsters, to primate, to squirrel monkeys, to Cynomolgus macaques, etc. etc. i could go on, but these are NOT humans, so the study means little toward human transmission'' etc.

i saw this headline the other day in the newspaper that some fool wrote ;

News Canada Humans likely resistant to disease found in deer: study By BOB WEBER, THE CANADIAN PRESS

Last Updated: 4th August 2009, 1:43am

http://www.edmontonsun.com/news/canada/2009/08/04/10353111-sun.html

U.S. study: deer infected with chronic wasting disease safe to eat

By Bob Weber, THE CANADIAN PRESS

http://www.edmontonsun.com/news/edmonton/2009/08/03/10349946.html

who ever wrote this story with the headlines will needlessly expose many to this disease, through nothing more than sensationalized media hype. and what about second hand transmission via friendly fire? have any of you ever looked at the real threat here from second, third, fourth passage etc. from TSE's. i keep trying to tell people that the consumption of cwd infected deer or elk might kill some, but it is the 'friendly fire', that is the bigger threat in my opinion. as the TSEs mutate, and or as they are passaged 1, 2, 3, times, they become more virulent. By changing what this study really showed, and sensationalizing the subject title with fiction, you saw what happened next, every other five and dime reporter picked up on the sensationalized subject, that was completely fabricated. I could care less who eats CWD tainted meat, but it's the after affects, the exposing millions, when they don't even know it, to possibly suffer the horrible consequences years, decades later, is not acceptable, and these five and dime news reporters are just as responsible for senselessly and needlessly exposing others with lazy reporting of sensationalized media reporting. then the other five and dimers pick up on it, and it spreads, just like CWD and BSe do, and you have every hunter out there now that will say that it's now o.k. to eat CWD infected deer and elk, because this study said that. THAT'S I NOT WHAT THIS STUDY SAID DAMN'T !!!

Science Daily (press release) - ‎22 hours ago‎

3, 2009) — Data from an ongoing multi-year study suggest that people who consume deer and elk with chronic wasting disease (CWD) may be protected from ...

http://www.sciencedaily.com/releases/2009/07/090730111152.htm

US study suggests deer infected with chronic wasting disease safe ... Bridge River Lillooet News - ?21 hours ago? A new study has added to evidence that meat from deer infected with chronic wasting disease - an infection similar to mad cow disease - is safe for humans ...

People Who Eat Deer And Elk With Chronic Wasting Disease May Avoid ...

Science Daily (press release) - ?22 hours ago? 3, 2009) — Data from an ongoing multi-year study suggest that people who consume deer and elk with chronic wasting disease (CWD) may be protected from ...

US study suggests deer infected with chronic wasting disease safe ...

Medicine Hat News - ?22 hours ago? EDMONTON - A new study has added to evidence that meat from deer infected with chronic wasting disease - an infection similar to mad cow disease - is safe ...

US study suggests deer infected with chronic wasting disease safe ...

Winnipeg Free Press - Bob Weber - ?Aug 3, 2009? EDMONTON - A new study has added to evidence that meat from deer infected with chronic wasting disease - an infection similar to mad cow disease - is safe ...

US study suggests deer infected with chronic wasting disease safe ...

CanadaEast.com - ?Aug 3, 2009? EDMONTON - A new study has added to evidence that meat from deer infected with chronic wasting disease - an infection similar to mad cow disease - is safe ...

The Canadian Press US study suggests deer infected with chronic wasting disease safe ... The Canadian Press - ?Aug 3, 2009? EDMONTON — A new study has added to evidence that meat from deer infected with chronic wasting disease - an infection similar to mad cow disease - is safe ...

http://news.google.com/news?um=1&ned=us&hl=en&q=chronic+wasting+disease&cf=all&scoring=d&start=10

o.k. let's look at what the study really said, and then the facts to date ;

For Immediate Release Wednesday, July 29, 2009

Species Barrier May Protect Macaques from Chronic Wasting Disease

Data from an ongoing multi-year study suggest that people who consume deer and elk with chronic wasting disease (CWD) may be protected from infection by an inability of the CWD infectious agent to spread to people. The results to date show that 14 cynomolgus macaques exposed orally or intracerebrally to CWD remain healthy and symptom free after more than six years of observation, though the direct relevance to people is not definitive and remains under study. Cynomolgus macaques often are used as research models of human disease because they are very close genetically to humans and are susceptible to several forms of human brain-damaging disease. Thus, it was decided to see whether exposure to CWD could induce disease in the macaques. The study appears online in the journal Emerging Infectious Diseases.

CWD is a type of brain-damaging disease known as a transmissible spongiform encephalopathy (TSE) or prion disease. CWD primarily affects deer, elk, and moose. Other TSE diseases include mad cow disease, or bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, and sporadic Creutzfeldt-Jakob disease (CJD) in humans. Humans are not susceptible to sheep scrapie, but BSE appears to have infected about 200 people, primarily in Europe in the 1990s. Those findings provided the rationale for the present CWD-macaque study, which began in 2003.

"We plan to continue this study for at least several more years because, although the risk to macaques so far appears to be low, we know that these diseases can take more than 10 years to develop," says Bruce Chesebro, M.D., chief of the Laboratory of Persistent Viral Diseases at Rocky Mountain Laboratories (RML) in Hamilton, Mont. RML is part of the National Institute of Allergy and Infectious Diseases (NIAID) of the National Institutes of Health (NIH). The RML group is leading the study with collaborators from the Colorado Division of Wildlife; State University of New York Downstate Medical Center; New York State Institute for Basic Research in Developmental Disabilities; American Red Cross; and the University of Wyoming.

The findings by the RML group support published field studies done by others in regions of Colorado and Wyoming where CWD is endemic. Between 1979 and 2001, there were no significant increases in human TSE diseases despite the likelihood that hunters in those areas were exposed to CWD through contact with infected animal tissue and contaminated hunting tools such as knives and saws. Extensive laboratory data also supports a human species barrier against CWD.

Notably, the RML study also included identical testing in squirrel monkeys, which are genetically less similar to humans than macaques. Of 15 squirrel monkeys exposed orally to CWD, two displayed disease symptoms 69 months after infection. Of 13 squirrel monkeys exposed intracerebrally to CWD, 11 displayed symptoms between 33 and 53 months after infection. In symptomatic animals, the presence of the CWD agent was confirmed in brain, spleen and lymph nodes.

The results in squirrel monkeys were not surprising because a study elsewhere in two squirrel monkeys yielded similar results. The study by the RML group was different, however, in that it tested oral exposure to CWD and also studied eight CWD samples from different areas of the country. The results in squirrel monkeys confirmed that disease progression in that species appears consistent with disease progression in deer and elk, where severe weight loss is nearly always present.

"The fact that the squirrel monkeys, like the deer and elk, suffered severe weight loss suggests that chronic wasting disease might affect a common region of the brain in different species," notes Dr. Chesebro.

NIAID conducts and supports research - at NIH, throughout the United States, and worldwide-to study the causes of infectious and immune-mediated diseases, and to develop better means of preventing, diagnosing and treating these illnesses. News releases, fact sheets and other NIAID-related materials are available on the NIAID Web site at http://www.niaid.nih.gov.

The National Institutes of Health (NIH) - The Nation's Medical Research Agency - includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov. -------------------------------------------------------------------------------- Reference: Race B et al. Susceptibilities of nonhuman primates to chronic wasting disease. Emerging Infectious Diseases. DOI: 10.3201/eid1509.090253.

http://www.nih.gov/news/health/jul2009/niaid-29.htm

what other science has shown ;

Program ID: Innovation Anthology #214 Program Date: 04/07/2009 Program Category: Health and Medicine, Prions, Social Sciences, Wildlife

CWD Potential Risk to Human Health Chronic wasting disease is an emerging epidemic among deer and elk on the prairies.

CWD is the cervid equivalent of mad cow disease. And according to Dr. Daniel Krewski, an expert in population health risk assessment, prion researchers are very worried about the impact of CWD on human health.

DR. DANIEL KREWSKI: And what would happen if that disease were to jump the species barrier. Hunters who consume deer and elk who may have CWD What we would like to do is get out ahead of the risk curve and try to anticipate and prevent the next transmission of a disease of this type to humans. So we are focusing on is it possible that CWD could transmit to humans? We will be hosting an expert group meeting in Ottawa where we are bringing over 10 international experts in this area to ask that question. Are there things that we can do to try to prevent that from happening as was the case with BSE turning into vCJD (variant Creutzfeld-Jacob disease) from the consumption of beef contaminated with the BSE (bovine spongiform encephalopathy) agent?

Dr. Krewski emphasizes the importance of investing now in preventive action to contain chronic wasting disease.

Thanks today to Canadian Institutes for Health Research.

http://www.innovationanthology.com/programs.php?id=229

Sunday, April 12, 2009

CWD UPDATE Infection Studies in Two Species of Non-Human Primates and one Environmental reservoir infectivity study and evidence of two strains

http://chronic-wasting-disease.blogspot.com/2009/04/cwd-update-infection-studies-in-two.html

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

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

http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summar

SNIP...END...TSS

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,? Michael W. Miller,? Pierluigi Gambetti,§ and Lawrence B. Schonberger*

*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; ?University of Wyoming, Laramie, Wyoming, USA; ?Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from:

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.

snip...full text ;

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Volume 12, Number 10-October 2006

Research

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Samantha MaWhinney,* W. John Pape,? Jeri E. Forster,* C. Alan Anderson,?§ Patrick Bosque,?¶ and Michael W. Miller#

*University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; ?Colorado Department of Public Health and Environment, Denver, Colorado, USA; ?University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Suggested citation for this article

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40-1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73-1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

snip... full text ;

http://0-www.cdc.gov.mill1.sjlibrary.org/ncidod/EID/vol12no10/06-0019.htm

full text ;

http://chronic-wasting-disease.blogspot.com/2006_12_01_archive.html

Monday, July 06, 2009

Prion infectivity in fat of deer with Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/07/prion-infectivity-in-fat-of-deer-with.html

CHRONIC WASTING DISEASE BLOG

http://chronic-wasting-disease.blogspot.com/

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

Thursday, July 23, 2009

UW Hospital warning 53 patients about possible exposure to rare brain disease

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

SEE THIS DAMNING VIDEO NOW AT THE BOTTOM OF THE BLOG BELOW ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html

NOW, you have to make your own mind up, but at least you have the rest of the facts. ...

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

also,

A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most

serious because of rapid horizontal spread and higher prevalence than BSE in

UK, up to 15% in some populations. Also may be a risk to humans - evidence

that it is not dangerous to humans is thin.

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

flounder — Mon, 13 Jul 2009 11:06:20 GMT

Journal of Wildlife Management 73(5):655-662. 2009 doi: 10.2193/2008-282

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

Christopher S. Jennelle1a, Michael D. Samuelb, Cherrie A. Noldenc, and Elizabeth A. Berkleyd

aDepartment of Forest and Wildlife Ecology, University of Wisconsin, 1630 Linden Drive, Madison, WI 53706, USA

bUnited States Geological Survey, Wisconsin Cooperative Wildlife Research Unit, University of Wisconsin, 1630 Linden Drive, Madison, WI 53706, USA

cDepartment of Forest and Wildlife Ecology, University of Wisconsin, 1630 Linden Drive, Madison, WI 53706, USA

dDepartment of Forest and Wildlife Ecology, University of Wisconsin, 1630 Linden Drive, Madison, WI 53706, USA

1E-mail: jennelle@wisc.edu

Abstract

Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy afflicting the Cervidae family in North America, causing neurodegeneration and ultimately death. Although there are no reports of natural cross-species transmission of CWD to noncervids, infected deer carcasses pose a potential risk of CWD exposure for other animals. We placed 40 disease-free white-tailed deer (Odocoileus virginianus) carcasses and 10 gut piles in the CWD-affected area of Wisconsin (USA) from September to April in 2003 through 2005. We used photos from remotely operated cameras to characterize scavenger visitation and relative activity. To evaluate factors driving the rate of carcass removal (decomposition), we used Kaplan–Meier survival analysis and a generalized linear mixed model. We recorded 14 species of scavenging mammals (6 visiting species) and 14 species of scavenging birds (8 visiting species). Prominent scavengers included American crows (Corvus brachyrhynchos), raccoons (Procyon lotor), and Virginia opossums (Didelphis virginiana). We found no evidence that deer consumed conspecific remains, although they visited gut piles more often than carcasses relative to temporal availability in the environment. Domestic dogs, cats, and cows either scavenged or visited carcass sites, which could lead to human exposure to CWD. Deer carcasses persisted for 18 days to 101 days depending on the season and year, whereas gut piles lasted for 3 days. Habitat did not influence carcass decomposition, but mammalian and avian scavenger activity and higher temperatures were positively associated with faster removal. Infected deer carcasses or gut piles can serve as potential sources of CWD prions to a variety of scavengers. In areas where surveillance for CWD exposure is practical, management agencies should consider strategies for testing primary scavengers of deer carcass material.

http://www.bioone.org/doi/abs/10.2193/2008-282

Friday, August 8, 2008

PS 76-59:

White-tailed deer carcass decomposition and risk of chronic wasting disease exposure to scavenger communities in Wisconsin

Chris S. Jennelle, Michael D. Samuel, Cherrie A. Nolden, and Elizabeth A. Berkley. University of Wisconsin

snip...

Chronic wasting disease (CWD) is an infectious transmissible spongiform encephalopathy (TSE) afflicting members of the family Cervidae, and causes neurodegeneration and ultimately death. While there have been no reports of natural cross-species transmission of CWD outside this group, we addressed the role of white-tailed deer (Odocoileus virginianus) carcasses as environmental sources of CWD in Wisconsin. Our objectives were to estimate rates of deer carcass and gut pile decomposition in the environment, characterize vertebrate scavenger communities, and quantify the relative activity of scavengers to determine CWD exposure risk. We placed 40 disease-free deer carcasses and nine gut piles in the CWD-affected area of Wisconsin from September to April in 2003 through 2005. We used photos from remotely operated cameras to characterize scavenger communities and relative activity. We used Kaplan-Meier survival analysis and a generalized linear mixed model to quantify the driving factors and rate of carcass removal (decomposition) from the environment.

Results/Conclusions

We recorded 14 species of scavenging mammals (six visiting species), and eight species of scavenging birds (14 visiting species). Prominent scavengers included American crows (Corvus brachyrhynchos), raccoons (Procyon lotor), and Virginia opossums (Didelphis virginiana). We found no evidence that deer directly consumed conspecific remains, although they visited them frequently. Domestic dogs (Canis familiaris), cats (Felis catus), and cows (Bos spp.) either scavenged or visited carcass sites, which could increase exposure risk of CWD to humans and human food supplies. Deer carcasses persisted for a median of 18 to 101 days, while gut piles lasted for a median of three days. Habitat did not influence carcass decomposition, but mammalian and avian scavenger activity and higher temperatures (proxy for microbial and arthropod activity) were associated with greater rates of carcass removal. Infected deer carcasses serve as environmental sources of CWD prions to a wide variety of mammalian and avian scavengers. Such sources of infectious material likely influence the maintenance and spread of CWD (in particular), and should be considered in the dynamics of other disease systems as well. Prudence would dictate the use of preemptive management strategies, and we highlight strategies for carcass disposal to mitigate the influence of carcasses as environmental sources of infectious diseases.

See more of PS 76 - Latebreaking: Disease and Epidemiology See more of Latebreakers

See more of The 93rd ESA Annual Meeting (August 3 -- August 8, 2008)

http://eco.confex.com/eco/2008/techprogram/P14681.HTM

snip... full text ;

Thursday, August 28, 2008

CWD TISSUE INFECTIVITY brain, lymph node, blood, urine, feces, antler velvet and muscle 2007 Annual Report

http://chronic-wasting-disease.blogspot.com/2008/08/cwd-tissue-infectivity-brain-lymph-node.html

7. Interspecies CWD transmission

Wild predators and scavengers are presumably feeding on CWD-infected carcasses. Skeletal muscle has been shown to harbor CWD prion infectivity [2], underscoring that other species will almost certainly be exposed to CWD through feeding. However, CWD has not been successfully transmitted by oral inoculation to species outside of the cervid family, suggestive of a strong species barrier for heterologous PrP conversion. Ferrets (family Mustelidae) can be infected with deer CWD after intracerebral (ic) but not oral exposure [5, 80]. Raccoons resisted even ic infection for up to 2 years thus far [24]. Mountain lion (Puma concolor) susceptibility to experimental feeding of CWD prions is currently under investigation (M. Miller and L. Wolfe, personal communication). Could wild rodents colonizing CWD- or scrapie-infected pastures serve as an environmental reservoir of prion infectivity? Interestingly, bank voles (Clethrionomys glareolus), are readily infected with CWD and sheep scrapie by intracerebral inoculation ([64]; U. Agrimi, unpublished data) and are considered as a potential reservoir for sheep scrapie [64]. Many vole species occur in North America [65, 83] and further research may determine whether voles enhance CWD or scrapie spread through environmental contamination.

see ;

Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

Thursday, December 25, 2008 Lions and Prions and Deer Demise

snip...

Greetings,

A disturbing study indeed, but even more disturbing, the fact that this very study shows the potential for transmission of the TSE agent into the wild of yet another species in the USA. Science has shown that the feline is most susceptible to the TSE agent. Will CWD be the demise of the mountain lions, cougars and such in the USA? How many have ever been tested in the USA? I recall there is a study taking place ;

Review A prion disease of cervids: Chronic wasting disease Christina J. Sigurdson et al ;

Mountain lion (Puma concolor) susceptibility to experimental feeding of CWD prions is currently under investigation (M. Miller and L. Wolfe, personal communication).

WHAT about multiple strains of CWD ?

0C7.04

North American Cervids Harbor Two Distinct CWD Strains

snip...

http://chronic-wasting-disease.blogspot.com/2008/12/lions-and-prions-and-deer-demise.html

Monday, January 05, 2009 CWD, GAME FARMS, BAITING, AND POLITICS

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html

NOT only muscle, but now fat of CWD infected deer holds infectivity of the TSE (prion) agent. ...TSS

Monday, July 06, 2009

Prion infectivity in fat of deer with Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/07/prion-infectivity-in-fat-of-deer-with.html

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

TSS

Monday, July 13, 2009

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/07/deer-carcass-decomposition-and.html

Infectious Prions in Pre-Clinical Deer and Transmission of Chronic Wasting Disease Solely by Environmental Exposure

flounder — Wed, 17 Jun 2009 11:33:00 GMT

PLoS ONE. 2009; 4(6): e5916. Published online 2009 June 16. doi: 10.1371/journal.pone.0005916. PMCID: PMC2691594

Copyright Mathiason et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Infectious Prions in Pre-Clinical Deer and Transmission of Chronic Wasting Disease Solely by Environmental Exposure

Candace K. Mathiason,1 Sheila A. Hays,1 Jenny Powers,2 Jeanette Hayes-Klug,1 Julia Langenberg,3 Sallie J. Dahmes,4 David A. Osborn,5 Karl V. Miller,5 Robert J. Warren,5 Gary L. Mason,1 and Edward A. Hoover1* 1Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, United States of America 2National Park Service, Fort Collins, Colorado, United States of America 3Wisconsin Department of Natural Resources, Madison, Wisconsin, United States of America 4WASCO Inc., Monroe, Georgia, United States of America 5Warnell School of Forestry and Natural Resources, University of Georgia, Athens, Georgia, United States of America Per Westermark, Editor Uppsala University, Sweden * E-mail: Edward.Hoover@ColoState.edu Conceived and designed the experiments: CKM EAH. Performed the experiments: CKM SAH JGP JHK. Analyzed the data: CKM GLM. Contributed reagents/materials/analysis tools: JL SJD DAO KVM RJW. Wrote the paper: CKM. Paper editing: EAH JGP JL. Received March 6, 2009; Accepted May 9, 2009.

Abstract

Key to understanding the epidemiology and pathogenesis of prion diseases, including chronic wasting disease (CWD) of cervids, is determining the mode of transmission from one individual to another. We have previously reported that saliva and blood from CWD-infected deer contain sufficient infectious prions to transmit disease upon passage into naïve deer. Here we again use bioassays in deer to show that blood and saliva of pre-symptomatic deer contain infectious prions capable of infecting naïve deer and that naïve deer exposed only to environmental fomites from the suites of CWD-infected deer acquired CWD infection after a period of 15 months post initial exposure. These results help to further explain the basis for the facile transmission of CWD, highlight the complexities associated with CWD transmission among cervids in their natural environment, emphasize the potential utility of blood-based testing to detect pre-clinical CWD infection, and could augur similar transmission dynamics in other prion infections.

snip...

Dicussion

Time interval to detection of CWD infection by tonsil biopsy The high transmission rate of CWD among cervids in their natural environment sets CWD apart from other prion diseases. The results of this study help provide a plausible basis for this facile transmission and extend our earlier findings [27] (Fig. 4) in demonstrating infectious prions in blood and saliva of pre-clinical CWD+ donors. The time from exposure to first detection of PrPCWD by tonsil biopsy was variable-as short as 6 months but as long as 18 months. We assume that the time until appearance of PrPCWD in tonsil is an underestimate due to the inherent variability in prion deposition kinetics [36] and the logistical limitations of tonsil biopsies, which require general anesthesia. The incubation periods prior to clinical CWD in our study were similar to those observed previously in experimental and naturally acquired infections [27], [37], [38]. While we can not exclude horizontal transmission from the first positive deer in each cohort, the timeframe for detection in the remaining deer (3 months) is less than half that which we have historically observed in deer inoculated orally with a brain homogenate from terminal CWD-infected deer [27], suggesting much earlier exposure to infectious prions.

Infectious prions in saliva of CWD+ deer

The presence of infectious CWD prions in saliva suggests the potential for disease transmission via grooming interactions, shared water sources and communal feeding grounds-especially in high density cervid populations, such as those that exist for some cervid species during the breeding season, at baiting sites, in captivity, and in low predation situations. Other investigators have detected the presence of the aberrant misfolded prion protein (PrPRES) in alimentary tract tissue, and have suggested saliva as a possible vehicle for prion transmission [27], [37], [39], [40]. While the volume of saliva used in this study was large, the results nevertheless provide evidence to support the above premise. Salivary dissemination of prions may not be limited to CWD. Prions associated with transmissible mink encephalopathy (TME) have been detected in the submandibular salivary gland of mink [41] and TME protease-resistant prion protein has been detected in the lamina propria of the oral cavity, taste buds and squamous epithelium of the tongue, and the vomeronasal organ and olfactory mucosa of infected hamsters [40]. Hamster-adapted scrapie agent has been found in the tongue and taste buds of prion-infected hamsters [42]. Vascellari et al reported the presence of the pathological prion protein in both major and minor salivary glands of naturally and experimentally infected sheep [43], and we have made similar observations in the olfactory mucosa of ferrets experimentally infected with CWD [44] and in the taste buds of deer (Haley, NJ, personal communication). The exact source of prions shed in saliva remains speculative; possible sources include centrifugal/retrograde passage from nerve fiber terminations in the oral-nasal mucosa, or from lymphoid cells emanating from infected tonsilar or other alimentary lymphoid tissues.

Infectious prions in blood of CWD+ deer

Blood-borne transmission of TSEs has long been feared, and the identification of a prion pathogen associated with blood-borne transmission has been pursued with disparate results [33], [45], [46]. Here we report the induction of CWD infection by a single blood transfusion from each of two pre-clinical CWD+ blood donors. This result is consistent with previous findings in substantiating the transmission of infectious prions by the blood of asymptomatic animal [27], [32] and human [28]-[30], [47], [48] donors, thus providing support for a subclinical hematogenous carrier state in TSE infections.

Direct detection of blood-borne PrPRES has been difficult. Saa et al were the first to use protein misfolding cyclic amplification assay (PMCA) [49], [50] to detect protease-resistant prion protein in the blood of asymptomatic scrapie-infected hamsters [51]. More recently, Thorne et al reported PMCA amplification of PrPSC from the blood of scrapie-infected sheep [52]. Continued efforts toward the development of sensitive, noninvasive, diagnostic tools are paramount. We are presently re-examining by serial PMCA the tissues of exposed but conventional PrPCWD test negative animals that may harbor infectious prions not manifested in the observation periods used in our CWD studies.

Hunter and colleagues [33], [34] provided the first evidence for blood-borne TSE transmission for bovine spongiform encephalopathy (BSE) and scrapie by transfusion of whole blood [33], [34] and buffy-coat white blood cells [34] from infected donor sheep to naïve sheep. Sparse but compelling evidence has accumulated for blood transmission of variant Creutzfeldt-Jakob Disease (vCJD) [28]-[30], [48] and PrPRES has been found in peripheral organs of some sporadic CJD patients [53], raising the possibility that peripheral distribution of PrPRES is not limited to vCJD. In an ongoing study of sixty-six individuals who received blood products from asymptomatic blood donors who later developed vCJD [54], three of the 66 blood transfusion recipients developed vCJD 6.5 to 8.5 years after receiving blood [28], [30], [48] and a fourth blood recipient died of causes unrelated to vCJD five years after receiving the blood donation. Upon autopsy of this individual, PrPRES was detected in lymphoid tissue but not brain, thus providing presumptive evidence for a case of subclinical infection [29]. Our findings with CWD further support the tenet that blood products from subclinical prion-infected individuals may transmit disease.

Additional cases of subclinical human prion disease may exist. While in vitro conversion studies have indicated an inefficient conversion of human PrP into a protease-resistant form [26], [55] and no evidence exists of CWD transmission to non-cervid species cohabitating with or on CWD contaminated environments [24], [56]-[58], it is reasonable to surmise that cross-species transmission of prions may require extenuating circumstances, i.e. origin of specific strains [59], [60], prolonged incubation time [61], and permissive genotypes [62]. At least two studies provide information bearing on these concerns. The first study, an ongoing longitudinal study to closely monitor 81 Americans who inadvertently consumed, or were exposed to, CWD+ venison at an upstate New York sportsman's feast, will conduct health evaluations of these individuals over the next six years [63]. The second, a retrospective study using western blot analysis of human tonsil and appendix samples collected in the United Kingdom (UK) to investigate possible exposure to the BSE agent, reported the detection of abnormal prion protein in three of 12,674 samples [64]. Mathematical modeling based on the results of this study predicts a minimum estimate of 3000 BSE infected people in the UK between 10-30 years of age. If this model is accurate, it predicts that 93% of these individuals could develop long-term subclinical infection [65].

Environmental sources of CWD infection

Previous studies have confirmed direct animal-to-animal contact-horizontal transmission-as an efficient mode for prion disease transmission [9], [66]. Moreover, Miller and colleagues [9], [67], [68] have provided substantial evidence for environmental contamination as a source of CWD infection. Our bioassay study inocula doses (50 ml saliva/deer), while efficient in establishing the infectious nature of saliva, are likely unrealistic doses to be acquired in a natural setting. To emulate a more feasible natural environment-associated dose, while negating direct animal-to-animal contact, we exposed naïve deer to repeated exposures to fomites from the suites of CWD-infected deer. The study design was meant to mirror the daily habits and movements of a deer in its natural setting in which it may return to an area contaminated with small amounts of infectious prions over time. Here we provide the first report that under controlled indoor conditions CWD-naïve deer can acquire infection by exposure to fomites from the environment of CWD-infected deer, supporting the findings of Miller et al in the natural environment [9], [67], [68], in demonstrating that there are sufficient infectious prions in bedding and water to transmit CWD. Efficient transmission, as evidenced by tonsillar lymphoid PrPCWD detection, was seen in as little as 15 months post initial exposure. These results are also consistent with the findings of Georgsson [69] and Miller [67] as part of their attempts to decontaminate areas heavily contaminated with scrapie and CWD. Animals reintroduced to these areas after decontamination developed clinical signs of prion disease within two years. The presence of infectious CWD prions in the environment therefore strongly suggests that natural prion infection occurs by routes additional to direct animal-to-animal contact. Based on the present and our previous findings [27], we speculate that saliva may harbor the greatest concentration of CWD prions available for horizontal transmission and environmental contamination, but recognize that other routes of excretion at lower concentration and greater volume still remain plausible.

Lack of detectable infectious prions in the urine and feces of CWD+ deer

Previous studies have postulated that environmental contamination by excreta from infected cervids seems the most plausible explanation for the dissemination of CWD [70], yet at 19 months pi we were not able to detect PrPCWD in the three deer inoculated with urine and feces. Our earlier report [27] indicated that 2 of 2 deer expressing the prnp gene G/S polymorphism at codon 96 remained negative 19 mo. pi. In the present study all three deer inoculated with urine and feces expressed the G/G polymorphism at prnp codon 96, which is associated with susceptibility to CWD infection [71]. We report no detection of PrPCWD in the obex or lymphoid tissues of deer with either G/G or G/S polymorphisms at 19 mo pi. Although both of our bioassay studies in deer have failed to transmit CWD infection by oral exposure to urine and feces from CWD-infected deer, these results must still be interpreted with caution in light of ongoing PMCA and cervid transgenic mouse intracerebral bioassay studies which suggest that very low concentrations of prions may be present in urine and feces of CWD+ cervids [72]-[74]. Perhaps an incubation time longer than 19 months is necessary for a detectable accumulation of lymphoid PrPCWD, or a larger dose of inoculum by the oral route is necessary for efficient passage of prions across the alimentary mucosa.

In summary, the results reported here reconfirm that blood and saliva are sources of infectious CWD prions, consistent with previous findings [27], and further support a mechanism for efficient CWD transmission in nature. We also show that infectious prions shed into the environment by CWD+ deer are sufficient to transmit the disease to naïve deer in the absence of direct animal-to-animal contact. These observations reinforce the exposure risk associated with body fluids, excreta, and all tissues from CWD+ cervids and suggest that similar dynamics may exist in other prion infections.

snip...

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2691594

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

Wednesday, March 18, 2009

Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay

http://chronic-wasting-disease.blogspot.com/2009/03/detection-of-cwd-prions-in-urine-and.html

Wednesday, January 07, 2009

CWD to tighten taxidermy rules Hunters need to understand regulations

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-to-tighten-taxidermy-rules-hunters.html

Thursday, December 25, 2008 Lions and Prions and Deer Demise

http://chronic-wasting-disease.blogspot.com/2008/12/lions-and-prions-and-deer-demise.html

Wednesday, March 18, 2009

Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

TSS

Tuesday, June 16, 2009

Infectious Prions in Pre-Clinical Deer and Transmission of Chronic Wasting Disease Solely by Environmental Exposure

http://chronic-wasting-disease.blogspot.com/2009/06/infectious-prions-in-pre-clinical-deer.html

CWD UPDATE Infection Studies in Two Species of Non-Human Primates and one Environmental reservoir infectivity study and evidence of two strains

flounder — Sun, 19 Apr 2009 20:00:25 GMT

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

CWD Infection Studies in Two Species of Non-Human Primates

Bruce Chesebro Laboratory of Persistent Virus Diseases, Rocky Mountain Laboratories, Hamilton, Montana USA 59840.

CWD is a TSE/prion disease present in wild and domestic cervid populations of North America. CWD from cervids might possibly spread to humans who hunt and eat these species and to domestic animals such as cattle, sheep or horses sharing the same habitat. Therefore, it is important to understand the potential for spread of CWD to other species. Laboratory experiments have shown that CWD does not cause disease in transgenic mice expressing human PrP, suggesting that humans and other primates might be resistant to this infection. However, earlier data from the laboratory of Richard Marsh found that squirrel monkeys could be infected by intracerebral CWD inoculation. We recently followed up this work extending it to studies of two primate species, squirrel monkeys and Cynomolgus macaques. We also compared intracerebral and oral routes of infection. To search for possible CWD variant strains we analyzed 8 different CWD pools obtained from wild or domestic elk, mule deer and white-tailed deer. The results of these experiments will be presented.

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf

J Virol. 2005 November; 79(21): 13794-13796. doi: 10.1128/JVI.79.21.13794-13796.2005. PMCID: PMC1262585

Copyright © 2005, American Society for Microbiology

Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus)

Richard F. Marsh,1? Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4* Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska 68178,4 Department of Veterinary Molecular Biology, Montana State University, Bozeman, Montana 597183 *Corresponding author. Mailing address: Department of Medical Microbiology and Immunology, Creighton University, 2500 California Plaza, Omaha, NE 68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail: jbartz@creighton.edu. ?Deceased. Received May 3, 2005; Accepted August 10, 2005. This article has been cited by other articles in PMC. Top AbstractChronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates.

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1262585

P1

THE ENVIRONMENT AS A RESERVOIR OF PRION INFECTIVITY

Aiken, Judd1,2, Chris Johnson4, Debbie McKenzie1,3 and Joel Pedersen5 1 Centre for Prions and Protein Folding Diseases, 2 Department of Agriculture, Food and Nutritional Sciences, 3 Department of Biological Sciences, University of Alberta, Edmonton, Alberta Canada, 4 National Wildlife Health Center, Madison, WI and 5 Department of Soil Sciences, University of Wisconsin, Madison

An environmental reservoir of prion infectivity has long been known to be a source of infection of sheep scrapie and likely plays an even more important role in the transmission of chronic wasting disease (CWD) in elk, deer and moose. Prion infectivity is extremely resistant to degradation, resulting in an environmental persistence of infectious agent. CWD is a contagious disease of free-ranging cervids. Infected deer and elk release infectious agent into the environment from body fluids and from diseased animal carcasses. The rapid expansion of CWD in North America represents a significant and continued environmental risk not only to cervids but to other species as well. Our work has demonstrated that prion protein, including PrPCWD, binds avidly to soil and soil components. Significantly, prion/soil binding enhances disease transmission suggesting that the soils, once contaminated with infectious prions, plays a critical role in maintaining and perpetuating prion infections.

III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf

P35

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.

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf

RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

___________________________________

PRODUCT

a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;

b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;

c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;

d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;

e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;

f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9;

CODE

Elk Meats with production dates of December 29, 30, and 31

RECALLING FIRM/MANUFACTURER

Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009.

Manufacturer: Noah's Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing.

REASON

Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD).

VOLUME OF PRODUCT IN COMMERCE

Unknown

DISTRIBUTION

NV, CA, TX, CO, NY, UT, FL, OK

___________________________________

http://www.fda.gov/bbs/topics/ENFORCE/2009/ENF01099.html

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

snip...

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST

our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.

http://www.jbc.org/

snip...

Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml

snip

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

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: [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask] Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

snip...

full text ;

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Wednesday, March 18, 2009 Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

Thursday, March 19, 2009

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

Wednesday, March 18, 2009

Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay

http://chronic-wasting-disease.blogspot.com/2009/03/detection-of-cwd-prions-in-urine-and.html

Thursday, March 26, 2009

HB 4214 - Texas: Relating to the business of taxidermy; providing penalties AND HELP PREVENT CWD

http://chronic-wasting-disease.blogspot.com/2009/03/hb-4214-texas-relating-to-business-of.html

Tuesday, January 27, 2009 Chronic Wasting Disease found in a farmed elk from Olmsted County ST. PAUL, Minn. FOR IMMEDIATE RELEASE: Monday, January 26, 2009

http://chronic-wasting-disease.blogspot.com/2009/01/chronic-wasting-disease-found-in-farmed.html

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

Monday, January 05, 2009

CWD, GAME FARMS, BAITING, AND POLITICS

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html

2008 CWD Laboratory Testing for Wild White-tailed Deer

http://www.michigan.gov/emergingdiseases/0,1607,7-186-25806-202922--,00.html

Wednesday, January 07, 2009

CWD to tighten taxidermy rules Hunters need to understand regulations

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-to-tighten-taxidermy-rules-hunters.html

Thursday, December 25, 2008 Lions and Prions and Deer Demise

http://chronic-wasting-disease.blogspot.com/2008/12/lions-and-prions-and-deer-demise.html

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

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?

http://www.neurology.org/cgi/eletters/60/2/176#535

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 Text

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 disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)-the relative of mad cow disease seen among deer and elk in the USA. Although his feverish ........

http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/fulltext

http://www.mdconsult.com/das/article/body/131155965-2/jorg=journal&source=&sp=13979213&sid=0/N/368742/1.html?issn=14733099

THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/

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. FREE FULL TEXT

http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406

TSS

Sunday, April 12, 2009

CWD UPDATE Infection Studies in Two Species of Non-Human Primates and one Environmental reservoir infectivity study and evidence of two strains

http://chronic-wasting-disease.blogspot.com/2009/04/cwd-update-infection-studies-in-two.html

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD) CDC WARNING 2009

flounder — Sat, 21 Mar 2009 12:59:39 GMT

I find this study most important.

some of us have been saying this for years, myself, i made a submission to the BSE Inquiry in England in 1998. At least they listened.

But i thought some of you would be interested in this.

I don't care really what anybody eats, and or, if you want to pour a gallon of urine on yourself, if it makes you feel good, to go for a hunt. whatever turns you on.

BUT, when you have the CDC finally come out with a warning like this after so many years of floundering, better late than never i suppose, but how many were exposed needlessly???

----- Original Message -----

From: "TERRY SINGELTARY" <flounder9@VERIZON.NET>

To: <CJD-L@LISTS.AEGEE.ORG>

Sent: Thursday, March 19, 2009 8:47 PM

Subject: [CJD-L] Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)

10.3201/eid1505.081458 Suggested citation for this article: Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis. 2009 May; [Epub ahead of print]

Chronic Wasting Disease Prions in Elk Antler Velvet

Rachel C. Angers,1 Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O'Rourke, Aru Balachandran, and Glenn C. Telling Author affiliations: University of Kentucky Medical Center, Lexington, Kentucky, USA (R.C. Angers, T.S. Seward, D. Napier, M. Green, G.C. Telling); Colorado State University, Fort Collins, Colorado, USA (E. Hoover, T. Spraker); US Department of Agriculture, Pullman, Washington, USA (K. O'Rourke); and Canadian Food Inspection Agency, Ottawa, Ontario, Canada (A. Balachandran) 1Current affiliation: MRC Laboratory of Molecular Biology, Cambridge, UK.

Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the apical skin layer covering the growing antler (antler velvet) by using CWD-susceptible transgenic mice and protein misfolding cyclic amplification. Our finding of prions in antler velvet of CWD-affected elk suggests that this tissue may play a role in disease transmission among cervids. Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. The fact that CWD prion incubation times in transgenic mice expressing elk prion protein are consistently more rapid raises the possibility that residue 226, the sole primary structural difference between deer and elk prion protein, may be a major determinant of CWD pathogenesis.

snip...

Discussion

The transmission of CWD prions in antler velvet from 2 naturally affected elk to mice in 2 Tg models demonstrates that this tissue contains low, but detectable, amounts of CWD prions. In addition, serial PMCA amplified otherwise undetectable levels of PrPSc in antler velvet. We characterized CWD prion infectivity by end-point titration. The .6 log i.c.ID50/g CWD prion titer estimated by this method contrasts with .9 log i.c.ID50/g titers of mouse-adapted scrapie prions in rodent brains (9) and .7.7.7 log i.c.ID50/g titers of BSE prions estimated by bioassay in transgenic mice (10,11). The linear relationship between dose and incubation time (12) provides an opportunity to estimate the level of prions in materials containing an unknown amount of infectivity. The attack rates of <100% after inoculation with antler velvet preparations from elk 01-0306 and 03-0306 and the failure to transmit disease from the remaining antler velvet samples suggest that CWD prion titers are close to, or at, the end point of the Tg(CerPrP)1536+/. bioassay. Although we are aware of the limitations of comparing levels of prions in tissues from different CWD-affected cervids, we estimate the end point of the CWD prion titration using D92 to be <3.5 log i.c.ID50 units. Other factors could also influence levels of infectivity in the 4 tested samples, e.g., the portion of the antler processed and the age of the antler when harvested. Histologic evaluation indicated that the velvet samples used in these transmission studies came from elk antlers in the early stages of seasonal growth (data not shown). Whether CWD prion titers in antler velvet vary according to the state of antler growth remains to be determined. Whether prion infectivity is derived from nervous system tissue, blood (13), or another component of velvet, is also unclear. Implications for Horizontal CWD Transmission and Human Exposure Our studies indicate that antler velvet represents a previously unrecognized source of CWD prions in the environment. Whereas oral transmission of rodent-adapted scrapie prions is known to be .5 orders of magnitude less efficient than transmission by intracerebral inoculation (14,15), the relative efficiency of oral CWD prion transmission is unknown. Multiple exposures

Page 7 of 17

to low levels of CWD prions in the environment (16,17), as well as increased infectivity when prions are bound to soil minerals (18), are factors that may influence transmission. The appearance of variant Creutzfeldt-Jakob disease in humans exposed to bovine spongiform encephalopathy (BSE) (19,20) and the demonstration of CWD prions in muscle (3) placed the human species barrier to CWD prions at the forefront of public health concerns. Our studies indicate that antler velvet represents an additional source for human exposure to CWD prions. Widely used in traditional Asian medicine to treat a variety of ailments including impotence, arthritis and high blood pressure, antler velvet can be readily purchased in caplet form and its usage has increased worldwide. Fortunately, to date there is no epidemiologic evidence for increased rates of CJD in the CWD-endemic region (Colorado, USA) (21,22). Also reassuring is the inefficient in vitro conversion of human PrP to protease-resistant PrP by CWD (23). Two studies have shown that CWD prions failed to induce disease in Tg mice expressing human PrP (24,25). However, the failure of BSE to be transmitted to Tg mice expressing human prion protein (HuPrP) was cited as early evidence for a BSE transmission barrier in humans (26); subsequent studies demonstrated a strong effect of the codon 129 polymorphism on transmissibility of BSE prions (27). To date, only mice expressing HuPrP with methionine at 129 have been challenged with CWD. In support of the argument that humans might be susceptible to CWD, intracerebral inoculation of squirrel monkeys produced disease after >30 months (28). Prion strain properties are also critical when considering the potential for interspecies transmission. The existence of multiple CWD strains has been suggested by several studies (4,25,29,30), but strain isolation and host range characterization have not been reported. Finally, it is worth considering that if CWD were to cross the species barrier into humans, this transmission source might not be recognized if the disease profile overlapped with one of the forms of sporadic CJD reported in North America.

Possible Role for Residue 226 in CWD Pathogenesis

Previous studies that demonstrated more rapid CWD prion incubation times in Tg mice expressing elk PrP (24,29) than in Tg(CerPrP)1536+/. mice (4) raised the possibility that the single amino acid difference at residue 226 between elk and deer PrP (5) may influence CWD pathogenesis (29). However, when the transmission characteristics of CWD isolates were directly compared in Tg mice expressing differing levels of deer or elk PrP, Tamguney et al.

Page 8 of 17

concluded that CWD incubation times were related solely to the level of PrP transgene expression (25). We compared CWD transmission in Tg(CerPrP-E226)5037+/. and Tg(CerPrP)1536+/. mice, which express PrP at levels .5-fold higher than PrP in wild type mouse brain (Figure 1A), and found that CWD transmission was consistently and substantially more rapid in Tg(CerPrP-E226)5037+/. mice. Our results appear compatible with more efficient CWD prion propagation by elk cellular prion protein (CerPrPC) containing E at residue 226 than by deer CerPrPC containing Q at this position. Consistent with this interpretation, despite 5-fold lower levels of transgene expression in Tg(CerPrP-E226)5029+/. than in Tg(CerPrP)1536+/. mice, mean incubation times of the D92 isolate were equivalent in these 2 lines (Table). Nonetheless, undetected differences in CerPrPC expression, for example in particular cell types, might result in more rapid disease and/or altered pathologic changes. The generation of transgenic mice expressing elk and deer coding sequences using gene replacement strategies would seem to be an excellent approach for resolving this issue. The different responses to CWD in Tg mice also appear to recapitulate aspects of CWD pathogenesis in the natural hosts. Previous limited comparative transmission studies indicated that CWD developed .25% more rapidly in orally challenged elk than deer (31). Although plaques were not detected in brains of CWD-affected elk, florid plaques have been observed in the brains of diseased deer (32,33). Similar differences in pathologic changes were observed in Tg(CerPrP-E226)5037+/. and Tg(CerPrP)1536+/. mice (Figure 4). Structural analyses suggest that residue 226 is located within a region of PrPC proposed to interact with a factor (34), possibly equivalent to the postulated protein X (35). Although mutation of the equivalent residue from Q to lysine (K) in epitope-tagged mouse PrP had no effect on PrPSc formation in transfected chronically infected ScN2A cells, the effects of the Q-to-E substitution were not assessed (36).

Acknowledgments We thank Dongyue Zhuang for excellent technical assistance. This work was supported by grants 2RO1NS040334-04 from the National Institute

http://www.cdc.gov/eid/content/15/5/pdfs/08-1458.pdf

snip...

1998 MY SUBMISSION TO THE BSE INQUIRY ENGLAND

Sender: "Patricia Cantos"

To: "Terry S Singeltary Sr. (E-mail)"

Subject: Your submission to the Inquiry

Date: Fri, 3 Jul 1998 10:10:05 +0100

3 July 1998 Mr Terry S Singeltary Sr. E-Mail: Flounder at wt.net Ref: E2979

Dear Mr Singeltary,

Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.

As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.

Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at http://www.bse.org.uk. Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on 0171 261 8332 should you have any queries.

Yours sincerely Patricia Cantos Families Team Leader Attachments TSS

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

-------- Original Message --------

Subject: re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

Date: Thu, 01 May 2003 16:04:35 -0400

From: "Marcia G Crosse"

To: CC: "Charles W Davenport" , "Carolyn Feis Korman" , "Martin Gahart"

Mr. Singletary,

We were informed by representatives of Metabolife, Inc. that Metabolife 356 was reformulated to remove bovine complex as an ingredient in the product, approximately September 2001. We did not independently verify the contents of the product.

Sincerely, Marcia Crosse Acting Director Health CarePublic Health and Science Issues U.S. General Accounting Office 441 G Street, N.W. Washington, D.C. 20548

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

-------- Original Message -------- Subject: Re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA'' Date: Thu, 01 May 2003 15:48:52 -0500 From: "Terry S. Singeltary Sr." To: Marcia G Crosse CC: Charles W Davenport , Carolyn Feis Korman , Martin Gahart References:

THANK YOU!

MIRACLES DO HAPPEN! ;-)

now all we need to do is;

snip......

one small step for man, one giant leap for mankind ;-)

however;

''We did not independently verify the contents of the product''

???

TSS

####### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ########

see history of mad cow in a pill ;

Thursday, March 19, 2009

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

Wednesday, March 18, 2009

Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay

http://chronic-wasting-disease.blogspot.com/2009/03/detection-of-cwd-prions-in-urine-and.html

RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

___________________________________

PRODUCT

a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;

b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;

c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;

d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;

e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;

f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9;

CODE

Elk Meats with production dates of December 29, 30, and 31

RECALLING FIRM/MANUFACTURER

Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009.

Manufacturer: Noah's Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing.

REASON

Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD).

VOLUME OF PRODUCT IN COMMERCE

Unknown

DISTRIBUTION

NV, CA, TX, CO, NY, UT, FL, OK

___________________________________

http://www.fda.gov/bbs/topics/ENFORCE/2009/ENF01099.html

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

snip...

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST

our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.

http://www.jbc.org/

snip...

Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml

snip

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

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: [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask] Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

snip...

full text ;

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Wednesday, March 18, 2009
Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK
RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

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

Saturday, January 24, 2009

Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html

Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

see full text ;

http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have Chronic Wasting

flounder — Mon, 09 Feb 2009 21:44:54 GMT

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 (February 9) Mon, 09 Feb 2009 14:25:00 -0600

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.

Recall -- Firm Press Release FDA posts press releases and other notices of recalls and market withdrawals from the firms involved as a service to consumers, the media, and other interested parties. FDA does not endorse either the product or the company.

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.

Exotic Meats USA purchased 1 case of Elk Tenderloins weighing 16.9 lbs. The Elk Tenderloin was sold from January 16 – 27, 2009. The Elk Tenderloins was packaged in individual vacuum packs weighing approximately 3 pounds each. A total of six packs of the Elk Tenderloins were sold to the public at the Exotic Meats USA retail store. Consumers who still have the Elk Tenderloins should return the product to Exotic Meats USA at 1003 NE Loop 410, San Antonio, TX 78209. Customers with concerns or questions about the Voluntary Elk Recall can call 1-800-680-4375. The safety of our customer has always been and always will be our number one priority.

Exotic Meats USA requests that for those customers who have products with the production dates in question, do not consume or sell them and return them to the point of purchase. Customers should return the product to the vendor. The vendor should return it to the distributor and the distributor should work with the state to decide upon how best to dispose. If the consumer is disposing of the product he/she should consult with the local state EPA office.

http://www.fda.gov/oc/po/firmrecalls/exoticmeats02_09.html

see recent studies ;

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

flounder — Mon, 09 Feb 2009 09:37:49 GMT

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

Saturday, January 24, 2009 Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report Research Project: Detection of Transmissible Spongiform Encephalopathy Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: Foodborne Contaminants Research

2008 Annual Report

1a.Objectives (from AD-416) We will develop highly sensitive diagnostic tests to detect transmissible spongiform encephalopathy (TSE) in livestock and wildlife animal species prior to the onset of clinical disease. We will also develop tests to confirm the presence or absence of TSE disease agents in ingredients of animal origin and decontaminated environments.

1b.Approach (from AD-416) The threat of BSE continues to affect export economics for US meat. Meanwhile scrapie continues to influence sheep profits and herd biosecurity, and CWD is spreading throughout North America. Thus U.S. animal industry stakeholders have identified detection of the TSE infectious agent (prions) as a priority biosecurity research issue essential for prevention of TSE diseases. We will build on our previous successes using mass spectrometry (MS) for high-sensitivity and specificity in detection of PrPsc as a marker for TSE infectivity in blood using a hamster scrapie model. We will also develop a novel PrP-null mouse strain and related myeloma cell culture system for production of monoclonal antibodies (MAb), which may be specific for PrPsc. We will then choose MS or MAb and validate our novel diagnostic for preclinical diagnosis of scrapie in sheep blood. Whereas MS and MAb methods rely on dissolved samples, contamination of agricultural products and environmental surfaces is associated with solid samples. So we will produce a cell culture based assay for TSE infectivity that is surface-adsorbed. After using the relatively convenient hamster model for early development, we will validate this technology for detection of scrapie in sheep brain on meat-and-bone meal and stainless steel. Replacing 5325-32000-007-00D (3/19/2008).

3.Progress Report At this point in the Project, in general, we are completing preliminary studies using our relatively convenient hamster and mouse models, and are starting to work with more agriculturally relevant sheep and deer tissues. We are finding the cervid tissues quite different from rodent tissues, in their requirements for sample workup (e.g., amount and quality of lipid and fiber) and in their expression of TSE infectivity and presence of markers. OSQR required us to establish a new collaboration with a reputable cell biologist, to assist with our cell-based scrapie assay. We now have a new MTA with Dr. Charles Weissmann (Scripps), under which we are sharing cell lines and laboratory protocols. We have completed one part of our speed congenics project to develop PrP-null (disease-resistant) mice for use in antibody generation. After conceiving a new procedure for immunogen enrichment, we performed experimental vaccination of these animals in our facilities. This project relates to NP103 Component 8: Prevention and control of transmissible spongiform encephalopathies. Problem statement 9A: Scrapie; 9B Chronic Wasting Disease (CWD); and 9C: Bovine Spongiform Encephalopathy (BSE).

4.Accomplishments 1. Proteinase K-free method for preparation of samples facilitates TSE blood assay.

The most widely used and regulatory approved methods for detection of Transmissible Spongiform Encephalopathy (TSE) contain a step in which the sample is subjected to digestion by a very strong enzyme, proteinase K, which degrades almost all proteins in the sample except for an Infectious isoform of the normal cellular prion protein, a prion (PrPsc). Although PrPsc has served well as a marker for brain disease, infectivity in the blood is mostly not proteinase K resistant. The proteinase K-free technique developed by ARS scientists in the Foodborne Contaminants Research Unit in Albany, CA will allow scientists to detect infectivity in blood. These efforts will lead to diagnostic tests that will save farmers and ranchers money and resources by allowing them to identify infected animals prior to purchase, sale or slaughter, and keep TSE-infected animals out of the US food supply. This accomplishment addresses NP103 Component 8: Prevention and Control of Transmissible Spongiform Encephalopathies; Problem Statement 9A: Scrapie; 9B: Chronic Wasting Disease (CWD); and 9C: Bovine Spongiform Encephalopathy (BSE).

2. Demonstrated conversion of a non-infectious normal cellular prion protein (PrP) into disease isoform in cell culture.

Although Transmissible Spongiform Encephalopathy (TSE) infectivity can be detected using animal models and mass spectroscopy, a cell culture system offers increased speed and throughput. ARS scientists in the Foodborne Contaminants Research Unit in Albany, CA developed conditions for growth and infection of existing cell cultures and cultures expressing transgenic PrP genes, observing conversion to the disease-associated PrPsc isoform. This method will be further developed to detect infectivity that is adsorbed onto surfaces, such as stainless steel and soil. These efforts will lead to diagnostic tests that will save farmers and ranchers money and resources by allowing them to identify infected areas and equipment before these areas or items can infect their animals. This accomplishment addresses NP103 Component 8: Prevention and Control of Transmissible Spongiform Encephalopathies; Problem Statement 9A: Scrapie; 9B: Chronic Wasting Disease (CWD); and 9C: Bovine Spongiform Encephalopathy (BSE).

5.Significant Activities that Support Special Target Populations None.

6.Technology Transfer Number of New Commercial Licenses Executed 1

Review Publications Bruederle, C.E., Hnasko, R.M., Kraemer, T., Garcia, R.A., Haas, M.J., Marmer, W.N., Carter, J.M. 2008. Prion infected Meat-and-Bone Meal is still infectious after biodiesel production. PLoS Pathogens. Available: http://dx.plos.org/10.1371/journal.pone.0002969

Onisko, B.C., Chen, N., Napoli, J. 2008. The Nuclear Transcription Factor RAR Associates with Neuronal RNA Granules and Suppresses Translation. Journal of Biological Chemistry. 283(30):20841-20847.

Sajnani, G., Pastrana, M.A., Dynin, I.A., Onisko, B.C., Requena, J.R. 2008. Insights on scrapie prion protein (prpsc) structure obtained by limited proteolysis and mass spectrometry. Journal of Molecular Biology. 382(2008):88-98.

http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=413072&showpars=true&fy=2008

snip...

see other relating studies, and concerns ;

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

TSS

Pathogenic prion protein is degraded by a manganese oxide mineral found in soils

flounder — Thu, 15 Jan 2009 11:11:14 GMT

Short Communication

Pathogenic prion protein is degraded by a manganese oxide mineral found in soils

Fabio Russo1,,, Christopher J. Johnson2,,, Chad J. Johnson2, Debbie McKenzie2, Judd M. Aiken2 and Joel A. Pedersen1

1 Department of Soil Science and Molecular and Environmental Toxicology Center, University of Wisconsin, Madison, WI, USA 2 Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin, Madison, WI, USA

Correspondence Joel A. Pedersen joelpedersen@wisc.edu

Prions, the aetiological agents of transmissible spongiform encephalopathies, exhibit extreme resistance to degradation. Soil can retain prion infectivity in the environment for years. Reactive soil components may, however, contribute to the inactivation of prions in soil. Members of the birnessite family of manganese oxides (MnO2) rank among the strongest natural oxidants in soils. Here, we report the abiotic degradation of pathogenic prion protein (PrPTSE) by a synthetic analogue of naturally occurring birnessite minerals. Aqueous MnO2 suspensions degraded the PrPTSE as evidenced by decreased immunoreactivity and diminished ability to seed protein misfolding cyclic amplification reactions. Birnessite-mediated PrPTSE degradation increased as a solution's pH decreased, consistent with the pH-dependence of the redox potential of MnO2. Exposure to 5.6 mg MnO2 ml-1 (PrPTSE : MnO2=1 : 110) decreased PrPTSE levels by 4 orders of magnitude. Manganese oxides may contribute to prion degradation in soil environments rich in these minerals.

These authors contributed equally to this work.

Present address: Dipartimento di Scienze del Suolo della Pianta dell'Ambiente e delle Produzioni Animali, Università Federico II, Portici (NA), Italy.

Present address: US Geological Survey, Biological Resources Division, National Wildlife Health Center, Madison, WI, USA.

Three supplementary figures are available with the online version of this paper.

http://vir.sgmjournals.org/cgi/content/abstract/90/1/275

Common soil mineral degrades the nearly indestructible prion Published: Thursday, January 15, 2009 - 08:42 in Health & Medicine Learn more about: brain ailments chemical disinfectants contaminated soil journal of general virology mad cow disease mineral In the rogues' gallery of microscopic infectious agents, the prion is the toughest hombre in town. Warped pathogens that lack both DNA and RNA, prions are believed to cause such fatal brain ailments as chronic wasting disease (CWD) in deer and moose, mad cow disease in cattle, scrapie in sheep and Creutzfeldt-Jakob disease in humans. In addition to being perhaps the weirdest infectious agent know to science, the prion is also the most durable. It resists almost every method of destruction from fire and ionizing radiation to chemical disinfectants and autoclaving, which reduce prion infectivity but fail to completely eliminate it.

Now, however, a team of Wisconsin researchers has found that a common soil mineral, an oxidized from of manganese known as birnessite, can penetrate the prion's armor and degrade the protein.

The new finding, which was reported earlier this month (Jan. 2) in the Journal of General Virology, is important because it may yield ways to decontaminate soil and other environments where prions reside.

"Prions are resistant to many of the conventional means of inactivating pathogens," says Joel Pedersen, a University of Wisconsin-Madison environmental chemist and the senior author of the new study. For example, autoclaving, a standard method for sterilization in the laboratory, will reduce the concentration of prions in solution, but fails to eliminate them altogether, as it does for virtually all other types of pathogens.

Because prions infect both wild and domesticated animals, the agent can contaminate barnyards and other areas where infected livestock are kept, as well as persist in natural environments where deer, elk and other animals can become infected by contact with contaminated soil.

Other studies have shown that prions can survive in the soil for at least three years, and that soil is a plausible route of transmission for some animals, Pedersen says. "We know that environmental contamination occurs in deer and sheep at least," he notes.

Prion reservoirs in the soil, Pedersen explains, are likely critical links in the chain of infection because the agent does not appear to depend on vectors - intermediate organisms like mosquitoes or ticks - to spread from animal to animal.

That the birnessite family of minerals possessed the capacity to degrade prions was a surprise, Pedersen says. Manganese oxides like birnessite are commonly used in such things as batteries and are among the most potent oxidants occurring naturally in soils, capable of chemically transforming a substance by adding oxygen atoms and stripping away electrons. The mineral is most abundant in soils that are seasonally waterlogged or poorly drained.

"A variety of manganese oxide minerals exist and one of the most common is birnessite. They are common in the sense that you find them in many soils, but in low concentrations," says Pedersen. "They are among the strongest oxidants in soil."

The new study, which was led by Fabio Russo of the University of Naples and Christopher J. Johnson of UW-Madison, was conducted on prions in solution in the laboratory. The group's working hypothesis, according to Pedersen, is that the mineral oxidizes the prion, a chemical process that can be seen in things like iron oxidizing to form rust or how cut pears and apples turn brown when exposed to oxygen.

The next step, Pedersen says, is to mix the mineral with contaminated soil to see if it has the same effect. If it does, birnessite may become a useful tool for cleaning up contaminated farmyards and other places where the prion may be concentrated in the soil.

"I expect that its efficacy would be somewhat diminished in soil," says Pedersen. "It's something we'll explore."

Source: University of Wisconsin-Madison

http://esciencenews.com/articles/2009/01/15/common.soil.mineral.degrades.nearly.indestructible.prion

Chronic Wasting Disease UW finding could be 'first step' in fight against chronic wasting disease prion By Mark Johnson of the Journal Sentinel

Posted: Jan. 14, 2009

Scientists at the University of Wisconsin-Madison might have found a chink in one of biology's most fearsome infectious agents: the prion, believed responsible for the family of brain-wasting diseases that includes Creutzfeldt-Jakob in humans and chronic wasting disease in deer.

Previous studies have showed that the mutant proteins called prions have the insidious ability to survive in soil and retain their ability to infect animals.

But Wisconsin researchers reported in the Journal of General Virology that an oxidized form of manganese called birnessite degraded prions in laboratory test tubes.

"It looks pretty good," said Patrick Bosque, a prion researcher and associate professor of neurology at Denver Health Medical Center. "They really do show that this does accelerate the degradation of this (prion) protein."

Chronic wasting disease was first detected in Wisconsin's deer herd in 2001, prompting considerable worry in a state where deer hunting holds a treasured place.

Other prion diseases are mad cow disease and its human variant and scrapie in sheep.

Prions can survive on metal surgical instruments. An autoclave, which uses steam and pressure to kill bacteria in minutes, requires a much longer period to eliminate prions.

Bosque cautioned that researchers still must test birnessite on animals to see whether it will protect them from contaminated soil. This is important because animals ingest soil - deliberately to supplement their mineral nutrition, but also by accident while feeding and grooming.

Very resilient Once they get inside soil, prions have proved a resilient foe.

Bosque said a case has been documented in which a field where sheep were infected with scrapie was kept empty for several years and then repopulated with sheep from a country that did not have scrapie.

Despite the precautions, the new sheep were infected with the disease.

"It's a first step," said Joel Pedersen, an environmental chemist at UW and one of the authors of the study. "It's promising, but we have much more to do."

Pedersen said researchers conducted their study on hamsters infected with a prion disease. Scientists isolated prions from the infected hamsters, put them in solution and introduced birnessite, a soft, dark brown or black mineral oxide. Scientists then monitored the decline of the prions.

In one scenario, Pedersen said, birnessite dramatically reduced the number of prions.

He said the scientists are preparing to follow up this work on two fronts. They will add prions to soil, mix in birnessite and then measure the prions to see whether the material is effective in decontaminating soil.

This, Pedersen stressed, is a technically challenging experiment.

Researchers also will expose prions to birnessite and then inject them into hamsters to learn whether treated prions will infect the animals.

The experiments in the new paper did not involve the deer illness, chronic wasting disease, but rather a different prion disease.

Pedersen said later experiments will focus on chronic wasting disease.

http://www.jsonline.com/news/wisconsin/37616469.html

Sunday, November 16, 2008 Resistance of Bovine Spongiform Encephalopathy (BSE) Prions to Inactivation

snip...

Published online before print March 14, 2000, 10.1073/pnas.050566797; Proc. Natl. Acad. Sci. USA, Vol. 97, Issue 7, 3418-3421, March 28, 2000

Medical Sciences New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

Paul Brown*,, Edward H. Rau, Bruce K. Johnson*, Alfred E. Bacote*, Clarence J. Gibbs Jr.*, and D. Carleton Gajdusek§

* Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, and Environmental Protection Branch, Division of Safety, Office of Research Services, National Institutes of Health, Bethesda, MD 20892; and § Institut Alfred Fessard, Centre National de la Recherche Scientifique, 91198 Gif sur Yvette, France

Contributed by D. Carleton Gajdusek, December 22, 1999

Abstract

One-gram samples from a pool of crude brain tissue from hamsters infected with the 263K strain of hamster-adapted scrapie agent were placed in covered quartz-glass crucibles and exposed for either 5 or 15 min to dry heat at temperatures ranging from 150°C to 1,000°C. Residual infectivity in the treated samples was assayed by the intracerebral inoculation of dilution series into healthy weanling hamsters, which were observed for 10 months; disease transmissions were verified by Western blot testing for proteinase-resistant protein in brains from clinically positive hamsters. Unheated control tissue contained 9.9 log10LD50/g tissue; after exposure to 150°C, titers equaled or exceeded 6 log10LD50/g, and after exposure to 300°C, titers equaled or exceeded 4 log10LD50/g. Exposure to 600°C completely ashed the brain samples, which, when reconstituted with saline to their original weights, transmitted disease to 5 of 35 inoculated hamsters. No transmissions occurred after exposure to 1,000°C. These results suggest that an inorganic molecular template with a decomposition point near 600°C is capable of nucleating the biological replication of the scrapie agent.

transmissible spongiform encephalopathy scrapie prion medical waste incineration

Introduction

The infectious agents responsible for transmissible spongiform encephalopathy (TSE) are notoriously resistant to most physical and chemical methods used for inactivating pathogens, including heat. It has long been recognized, for example, that boiling is ineffective and that higher temperatures are most efficient when combined with steam under pressure (i.e., autoclaving). As a means of decontamination, dry heat is used only at the extremely high temperatures achieved during incineration, usually in excess of 600°C. It has been assumed, without proof, that incineration totally inactivates the agents of TSE, whether of human or animal origin. It also has been assumed that the replication of these agents is a strictly biological process (1), although the notion of a "virus" nucleant of an inorganic molecular cast of the infectious -pleated peptide also has been advanced (2). In this paper, we address these issues by means of dry heat inactivation studies.

full text;

http://www.pnas.org/cgi/content/full/97/7/3418

infectivity surviving ashing to 600*C is (in my opinion) degradable but infective. based on Bown & Gajdusek, (1991), landfill and burial may be assumed to have a reduction factor of 98% (i.e. a factor of 50) over 3 years. CJD-infected brain-tissue remained infectious after storing at room-temperature for 22 months (Tateishi et al, 1988). Scrapie agent is known to remain viable after at least 30 months of desiccation (Wilson et al, 1950). and pastures that had been grazed by scrapie-infected sheep still appeared to be contaminated with scrapie agent three years after they were last occupied by sheep (Palsson, 1979).

http://europa.eu.int/comm/food/fs/sc/ssc/out58_en.pdf

PAUL BROWN SCRAPIE SOIL TEST

http://www.bseinquiry.gov.uk/files/sc/seac07/tab03.pdf

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

PMID: 8006664 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

LANCET

Volume 351, Number 9110 18 April 1998 [Previous] [Next]

BSE: the final resting place

How to dispose of dangerous waste is a question that has vexed the human race for hundreds of years. The answer has usually been to get it out of sight--burn it or bury it. In Periclean Athens, victims of the plague were incinerated in funeral pyres; in 14th century Venice, a law stipulated that Black Death corpses should be buried to a minimum depth of 5 feet; and now, as the 20th century draws to a close, we are challenged by everything from industrial mercury to the smouldering reactors of decommissioned atomic submarines.

The Irish Department of Agriculture will convene an expert panel on April 27-29 to discuss the disposal of tissues from animals with bovine spongiform encephalopathy (BSE). Proper disposal of tissues from infected cattle has implications for both human and animal safety. Safety for human beings is an issue because there is now unassailable if still indirect evidence that BSE causes infections in man in the form of "new variant" Creutzfeld-Jakob disease (nvCJD).1-3 Safety for animals is also an issue because BSE-affected cattle could possibly transmit disease to species other than cattle, including sheep, the species that was almost surely the unwitting source of the BSE epidemic.

The first matter to consider is the distribution of infectivity in the bodies of infected animals. The brain (and more generally, the central nervous system) is the primary target in all transmissible spongiform encephalopathies (TSE), and it contains by far the highest concentration of the infectious agent. In naturally occuring disease, infectivity may reach levels of up to about one million lethal doses per gram of brain tissue, whether the disease be kuru, CJD, scrapie, or BSE. The infectious agent in BSE-infected cattle has so far been found only in brain, spinal cord, cervical and thoracic dorsal root ganglia, trigeminal ganglia, distal ileum, and bone marrow.4 However, the much more widespread distribution of low levels of infectivity in human beings with kuru or CJD, and in sheep and goats with scrapie, suggests that caution is advisable in prematurely dismissing as harmless other tissues of BSE-infected cattle.

A second consideration relates to the routes by which TSE infection can occur. Decades of accumulated data, both natural and experimental, have shown clearly that the most efficient method of infection is by direct penetration of the central nervous system; penetration of peripheral sites is less likely to transmit disease. Infection can also occur by the oral route, and the ingestion of as little as 1 g of BSE brain tissue can transmit disease to other cattle.5 Infection by the respiratory route does not occur (an important consideration with respect to incineration), and venereal infection either does not occur or is too rare to be detected.

How can tissue infectivity be destroyed before disposal? The agents that cause TSE have been known almost since their discovery to have awesome resistance to methods that quickly and easily inactivate most other pathogens. Irradiation, chemicals, and heat are the three commonest inactivating techniques. Irradiation has proved entirely ineffective, and only a handful of a long catalogue of chemicals have produced more than modest reduction in infectivity. The most active of these are concentrated solutions of sodium hypochlorite (bleach) or sodium hydroxide (lye). As for heat, even though the agent shares with most other pathogens the feature of being more effectively damaged by wet heat than by dry heat, boiling has little effect, and steam heat under pressure (autoclaving) at temperatures of 121ºC is not always sterilising. To date, the most effective heat kill requires exposure of infectious material to steam heat at 134ºC for 1 h in a porous-load autoclave.6 Exposure to dry heat even at temperatures of up to 360ºC for 1 h may leave a small amount of residual infectivity.7 The standard method of incineration, ***heating to about 1000ºC for at least several seconds, has been assumed to achieve total sterilisation, but needs experimental verification in the light of suggestions that rendered tissue waste might find some useful purpose as a source of heating fuel.

Thus, TSE agents are very resistant to virtually every imaginable method of inactivation, and those methods found to be most effective may, in one test or another, fail to sterilise. It seems that even when most infectious particles succumb to an inactivating process, there may remain a small subpopulation of particles that exhibit an extraordinary capacity to withstand inactivation, and that, with appropriate testing, will be found to retain the ability to transmit disease. Also, almost all available inactivation data have come from research studies done under carefully controlled laboratory conditions, and it is always difficult to translate these conditions to the world of commerce. Even when the data are applied in the commercial process, the repetitive nature of the process requires vigilance in quality control and inspection to ensure adherence to its regulations.

The final issue that must be addressed is the "lifespan" of the infectious agent after disposal if it has been only incompletely inactivated beforehand. Given the extraordinary resistance of the agent to decontamination measures, the epidemiological and experimental evidence indicating that TSE agents may endure in nature for a long time should come as no surprise. The first real clue to this possibility came from the Icelandic observation that healthy sheep contracted scrapie when they grazed on pastures that had lain unused for 3 years after having been grazed by scrapie-infected sheep.8

Support for this observation was obtained from an experiment in which scrapie-infected brain material was mixed with soil, placed in a container, and then allowed to "weather" in a semi-interred state for 3 years.9 A small amount of residual infectivity was detected in the contaminated soil, and most of the infectivity remained in the topmost layers of soil, where the tissue had originally been placed--in other words, there had been no significant leaching of infectivity to deeper soil layers.

It is therefore plausible for surface or subsurface disposal of TSE-contaminated tissue or carcasses to result in long-lasting soil infectivity. Uncovered landfills are a favourite feeding site for seagulls, which could disperse the infectivity.10 Other animals might do likewise, and if the landfill site were later used for herbivore grazing, or tilled as arable land, the potential for disease transmission might remain. A further question concerns the risk of contamination of the surrounding water table, or even surface waste-water channels, by effluents and discarded solid waste from treatment plants.

A reasonable conclusion from existing data is that there is a potential for human infection to result from environmental contamination by BSE-infected tissue residues. The potential cannot be quantified because of the huge number of uncertainties and assumptions that attend each stage of the disposal process.

On the positive side, spongiform encephalopathy can be said to be not easily transmissible. Although the level of infectivity to which creatures are exposed is not known, it is probably very low, since sheep that die from scrapie, cattle that die from BSE, and human beings who die from nvCJD represent only a small proportion of their respective exposed populations.

Whatever risk exists is therefore extremely small, but not zero, hence all practical steps that might reduce the risk to the smallest acceptable level must be considered. What is practical and what is acceptable are concepts that will be hammered out on the anvil of politics: scientific input, such as it is, already waits in the forge. A fairly obvious recommendation, based on the science, would be that all material that is actually or potentially contaminated by BSE, whether whole carcasses, rendered solids, or waste effluents, should be exposed to lye and thoroughly incinerated under strictly inspected conditions. Another is that the residue is buried in landfills to a depth that would minimise any subsequent animal or human exposure, in areas that would not intersect with any potable water-table source. Certainly, it has been, and will continue to be, necessary in many instances to accept less than the ideal.

Paul Brown

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892, USA

1 Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 921-25 [PubMed].

2 Bruce M, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997: 389: 498-501.

3 Collinge J, Sidle KCL, Heads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].

4 Wells GAH, Hawkins SAC, Green RB, et al. Preliminary observations on the pathogenesis of experimental bovine spongiform encephalopathy (BSE): an update. Vet Rec 1998; 142: 103-06 [PubMed].

5 Collee JG, Bradley R. BSE: a decade on--part 2. Lancet 1997; 349: 715-21 [PubMed].

6 Taylor DM. Exposure to, and inactivation of, the unconventional agents that cause transmissible degenerative encephalopathies. In: Baker HF, Ridley RM, eds. Methods in molecular medicine: prion diseases. Totawa NJ: Humana Press, 1996: 105-18.

7 Brown P, Liberski PP, Wolff A, Gajdusek DC. Resistance of scrapie infectivity to steam autoclaving after formaldehyde fixation and limited survival after ashing at 360°C: practical and theoretical implications, J Infect Dis 1990; 161: 467-72 [PubMed].

8 Palsson PA. Rida (scrapie) in Iceland and its epidemiology. In: Prusiner SB, Hadlow WJ, eds. Slow transmissible diseases of the nervous system, vol I. New York: Academic Press, 1979: 357-66.

9 Brown P, Gajdusek DC. Survival of scrapie virus after 3 years' interment. Lancet 1991; 337; 269-70.

10 Scrimgoeur EM, Brown P, Monaghan P. Disposal of rendered specified offal. Vet Rec 1996; 139: 219-20 [PubMed].

http://www.thelancet.com/newlancet/sub/issues/vol351no9110/body.commentary1146.html

1.2 Visual Impact

It is considered that the requirement for any carcass incinerator design would be to ensure that the operations relating to the reception, storage and decapitation of diseased carcasses must not be publicly visible and that any part of a carcass could not be removed or interfered with by animals or birds. ...

http://www.bseinquiry.gov.uk/files/yb/1989/04/03006001.pdf

88. Natural decay: Infectivity persists for a long time in the environment. A study by Palsson in 1979 showed how scrapie was contracted by healthy sheep, after they had grazed on land which had previously been grazed by scrapie-infected sheep, even though the land had lain fallow for three years before the healthy sheep were introduced. Brown also quoted an early experiment of his own (1991), where he had buried scrapie-infected hamster brain and found that he could still detect substantial infectivity three years later near where the material had been placed. 89. Potential environmental routes of infection: Brown discusses the various possible scenarios, including surface or subsurface deposits of TSE-contaminated material, which would lead to a build-up of long-lasting infectivity. Birds feeding on animal remains (such as gulls visiting landfill sites) could disperse infectivity. Other animals could become vectors if they later grazed on contaminated land. "A further question concerns the risk of contamination of the surrounding water table or even surface water channels, by effluents and discarded solid wastes from treatment plants. A reasonable conclusion is that there is a potential for human infection to result from environmental contamination by BSE-infected tissue residues. The potential cannot be quantified because of the huge numbers of uncertainties and assumptions that attend each stage of the disposal process". These comments, from a long established authority on TSEs, closely echo my own statements which were based on a recent examination of all the evidence. 90. Susceptibility: It is likely that transmissibility of the disease to humans in vivo is probably low, because sheep that die from scrapie and cattle that die from BSE are probably a small fraction of the exposed population. However, no definitive data are available. 91. Recommendations for disposal procedures: Brown recommends that material which is actually or potentially contaminated by BSE should be: 1) exposed to caustic soda; 2) thoroughly incinerated under carefully inspected conditions; and 3) that any residue should be buried in landfill, to a depth which would minimise any subsequent animal or human exposure, in areas that would not intersect with any potable water-table source. 92. This review and recommendations from Brown have particular importance. Brown is one of the world's foremost authorities on TSEs and is a senior researcher in the US National Institutes of Health (NIH). It is notable that such a respected authority is forthright in acknowledging the existence of potential risks, and in identifying the appropriate measures necessary to safeguard public health. Paper by SM Cousens, L Linsell, PG Smith, Dr M Chandrakumar, JW Wilesmith, RSG Knight, M Zeidler, G Stewart, RG Will, "Geographical distribution of variant CJD in the UK (excluding Northern Ireland)". Lancet 353:18-21, 2 nd January 1999 93. The above paper {Appendix 41 (02/01/99)} (J/L/353/18) examined the possibility that patients with vCJD (variant CJD) might live closer to rendering factories than would be expected by chance. All 26 cases of vCJD in the UK with onset up to 31 st August 1998 were studied. The incubation period of vCJD is not known but by analogy with other human TSEs could lie within the range 5-25 years. If vCJD had arisen by exposure to rendering products, such exposure might plausibly have occurred 8-10 years before the onset of symptoms. The authors were able to obtain the addresses of all rendering plants in the UK which were in production in 1988. For each case of vCJD, the distance from the place of residence on 1st January 1998 to the nearest rendering plant was calculated................SNIP...END

http://www.bse.org.uk/files/ws/s019b.pdf

PLoS ONE. 2008; 3(8): e2969. Published online 2008 August 13. doi: 10.1371/journal.pone.0002969. PMCID: PMC2493038

Copyright This is an open-access article distributed under the terms of the Creative Commons Public Domain declaration which stipulates that, once placed in the public domain, this work may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

Cathrin E. Bruederle,1* Robert M. Hnasko,1 Thomas Kraemer,2 Rafael A. Garcia,3 Michael J. Haas,3 William N. Marmer,3 and John Mark Carter1 1USDA-ARS WRRC, Foodborne Contaminants Research Unit, Albany, California, United States of America 2Forensic Toxicology, Institute of Legal Medicine, Saarland University, Homburg/Saar, Germany

3USDA-ARS ERRC, Fats, Oils and Animal Coproducts Research Unit, Wyndmoor, Pennsylvania, United States of America Neil Mabbott, Editor

University of Edinburgh, United Kingdom * E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000089/!x-usc:mailto:cathrin.bruederle@gmail.com

Conceived and designed the experiments: CEB RMH WNM JMC. Performed the experiments: CEB RMH TK. Analyzed the data: CEB TK JMC. Contributed reagents/materials/analysis tools: CEB RMH TK RAG MJH JMC. Wrote the paper: CEB. Received April 21, 2008; Accepted July 24, 2008.

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2493038

P04.08 Environmental Persistence of TSEs - Extraction of PrPSc from Soil Smith, A; Fernie, Karen; Somerville, R Neuropathogenesis Unit, UK Background: There are concerns about the potential spread of transmissible spongiform encephalopathies (TSEs) by environmental routes following, for example, burial of infected carcasses or the disposal of waste water. The extent to which TSE infectivity survives or is disseminated within soil and soil water is unclear as is the likelihood of ensuing infection. Aim: As part of this environmental project, soil samples are being collected from lysimeters containing either infected bovine heads or boluses of infectivity. The aim of this experiment is to devise a method for the extraction of PrPSc from soil and examines the interaction between soil and its components and TSE infectivity. Methods: Samples from two soil types (clay and sandy loam) were spiked with known amounts of TSE infected brain homogenate and subjected to various extraction methods including combinations of freeze/thaw, boiling, sonication and mixing with various solvents and detergents. Any recovery was determined on western blot using PrPSc as a surrogate marker for the presence of TSE infectivity. Results: These experiments have shown that PrPSc binds strongly to both sandy and clay soil, and to pure sand (quartz). Elution from quartz and the soils was only achieved in the presence of the detergent sarkosyl, and in the case of clay soil, satisfactory elution was only achieved if PrPSc was digested with proteinase K. This finding suggests that components in clay soil may bind differently to PrP than those of sandy soil, and that the N-terminal domain of PrP is involved in this binding. Conclusion: These results form the basis of a method for the extraction of PrPSc from soil and will be used to assay samples from a large scale lysimeter experiment. Samples testing positive for the presence of PrPSc will be selected for bioassay in mice. Results to date suggest that TSE infectivity may bind strongly to soil components and could therefore persist in the environment for long periods of time.

P04.61

Survival of PrPSc during Simulated Wastewater Treatment Processes

Pedersen, J1; Hinckley, G1; McMahon, K2; McKenzie, D3; Aiken, JM3 1University of Wisconsin, Soil Science/Civil and Environmental Engineering, USA; 2University of Wisconsin, Civil and Environmental Engineering, USA; 3University of Wisconsin, Comparative Biosciences, USA

Concern has been expressed that prions could enter wastewater treatment systems through sewer and/or septic systems (e.g., necropsy laboratories, rural meat processors, private game dressing) or through leachate from landfills that have received TSE-contaminated material. Prions are highly resistant to degradation and many disinfection procedures raising concern that they could survive conventional wastewater treatment. Here, we report the results of experiments examining the partitioning and survival of PrPSc during simulated wastewater treatment processes including activated and mesophilic anaerobic sludge digestion. We establish that PrPSc can be efficiently extracted from activated and anaerobic digester sludges with 1% sodium dodecyl sulfate, 10% sodium undecyl sulfate, and 1% sodium N-lauryl sarcosinate. Activated sludge digestion does not result in significant degradation of PrPSc. The protein partitions strongly to the activated sludge solids and is expected to enter biosolids treatment processes. A large fraction of PrPSc survived simulated mesophilic anaerobic sludge digestion. Our results suggest that if prions were to enter municipal waste water treatment systems, most of the agent would partition to activated sludge solids, survive mesophilic anaerobic digestion, and be present in treated biosolids. Land application of biosolids containing prions could represent a route for their unintentional introduction into the environment. Our results argue for excluding inputs of prions to municipal wastewater treatment facilities that would result in unacceptable risk of prion disease transmission via contaminated biosolids.

P04.71 Oral Transmission of Prion Disease Is Enhanced by Binding to Soil Particles Johnson, C; Pedersen, J; Chappell, R; McKenzie, D; Aiken, J University of Wisconsin - Madison, USA A long-unanswered question in prion biology is how certain transmissible spongiform encephalopathies (TSEs), such as sheep scrapie and cervid chronic wasting disease, spread from animal to animal. Anecdotal evidence and controlled field experiments have suggested the presence of an environmental TSE reservoir. We, and others, have speculated that soil may harbor TSE agent in the environment and allow its transfer to naïve hosts. TSE infectivity can persist in soil for years, and we previously demonstrated that the disease-associated form of the prion protein binds to soil particles and that prions adsorbed to the common soil mineral montmorillonite (Mte) retain infectivity following intracerebral inoculation. We assessed the oral infectivity of Mte- and soil-bound prions and found that prions bound to Mte are orally bioavailable and that, unexpectedly, binding to Mte significantly enhances disease penetrance and reduces incubation period relative to unbound agent. Cox proportional hazards modelling revealed that across the doses of TSE agent tested, Mte increased the effective infectious titer by a factor of 680 relative to unbound agent. Oral exposure to Mte-associated prions led to TSE development in experimental animals even at doses too low to produce clinical symptoms in the absence of the mineral. We tested the oral infectivity of prions bound to three whole soils differing in texture, mineralogy and organic carbon content, and found soil-bound prions to be orally infectious. Two of the three soils increased oral transmission of disease, and the infectivity of agent bound to the third soil was equivalent to that of unbound agent. Enhanced infectivity of soilbound prions may explain the environmental transmission of some TSEs despite the presumably low levels shed into the environment.

P04.104 Survival of Prion Proteins in Environmental Matrices Maluquer de Motes, C1; Torres, JM2; Pumarola, M3; Girones, R1 1University of Barcelona, Spain; 2Centro de Investigacion en Sanidad Animal, Spain; 3Autonomous University of Barcelona, Spain Several publications have suggested the environment as a possible route of transmission, especially for sheep scrapie and cervid Chronic Wasting Disease (CWD). The role of the environment as a reservoir for these disorders is difficult to prove and faces a considerable lack of information. In this work, different methodologies have been developed to evaluate the survival and inactivation of TSE agents in environmental matrices. Different slaughterhouse and urban sewage samples were spiked with diverse strains of either scrapie or BSE agents and kept under controlled conditions for extended periods of time. Aliquots of every experiment were sequentially collected and concentrated according to a methodology specifically selected for each type of matrix. Sensitivity of the methods developed was estimated among 2-10 fÊg of infected tissue. PrPres was finally detected by western blot. Films were then transformed into digital pictures, signal intensities were quantified and regression models were computed. According to the results obtained, scrapie agent showed higher stability than BSE in all the environments studied. However, no significant differences were observed among mouse-passaged scrapie strains and sheep scrapie. The regression models provided t90 and t99 values (times of incubation necessaries for 90% and 99% reduction of PrPres levels). In urban sewage, i.e., t99 was estimated as about 50 and 22 days for scrapie and BSE respectively. In general, the effect of the matrix was clearly observed in all the experiments, showing up to a 6-8 fold higher reduction of PrPres levels in comparison to PBS controls. As some of the inocula were titrated in terms of infectious doses, we approximated the decay of PrPres levels to the reduction of infectivity for both agents. In slaughterhouse wastewater, i.e., two-log reduction was observed for both agents after 30-35 days of incubation. Data on infectivity will be confirmed by a series of bioassay experiments.

P04.125 Environmental Persistence of TSE Infectivity: Field Studies Fernie, K; Smith, A; Somerville, R Neuropathogenesis Unit, Roslin Institute, UK Background: There is concern about the consequences of contamination of the environment with TSE infectivity. Infectivity may enter the environment by various routes, persist in the ground and spread from the original source to contaminate an extended area and groundwater. Aims: We are studying this problem by addressing the following questions: 1. Does infectivity with some containment (e.g. in a carcass) survive in the carcass over time; 2. Does infectivity without containment survive, and is it disseminated into the surrounding soil and water? 3. Do the environmental conditions, e.g. soil type and pH, affect the survival and/or transport of infectivity through soil? Methods: To address these questions, we are performing two field experiments (with appropriate containment) each using two soil types. Air temperature, rainfall, soil temperature and moisture content are being monitored. In one experiment a series of 10 bovine heads have been spiked with the BSE derived TSE strain 301V and buried in the two soils, contained within individual lysimeters, for exhumation and analysis at yearly intervals. Rainwater flowing through and collected as groundwater is also being analysed. In the second experiment a bolus of infected brain is buried at the centre of two 3 meter diameter lysimeters and soil samples taken from them at regular intervals. Water flow-through is also analysed. Results: To date, the first two bovine heads have been exhumed and the surrounding soil sampled. Both of the exhumed heads were apparently largely decomposed but on examination of the brain cavity were found to contain significant amounts of brain tissue. These have been sampled and are presently being analysed. The soil samples taken from around the heads and five sets of core samples taken from the soil surrounding the buried brain in the two large lysimeters are presently being analysed for PrPSc, the abnormal protein associated with the TSEs and for infectivity. Water samples have also been collected for analyses. Discussion: We will use the acquired data to build a predictive model of TSE behaviour in the environment which will inform future risk assessments.

snip...end....NEUROPRION 2007...TSS

Public release date: 11-Aug-2008

Contact: Dr. Björn Seidel mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000089/!x-usc:mailto:bjoern.seidel@ime.fraunhofer.de 49-297-230-2330 Fraunhofer-Gesellschaft

Resistant prions

A flock of sheep at pasture - a seemingly idyllic scene. But appearances can be deceptive: If the animals are suffering from scrapie, entire flocks may perish. Scrapie is an infectious disease in which prions destroy the animal's brain, rather like BSE. The brain becomes porous, the sheep lose their orientation, they suffer from strong itching sensations and scrape off their fleece. Eventually, the infected animals die.

It is difficult to contain the disease - all too often, scrapie will break out again on the same farm several months or years after it has apparently been eradicated. Are the prions transmitted not only by direct contact, but also by the environment - perhaps by the pastures? How long do prions that get into the pasture via the saliva and excrements of the sick animals, persist in the ground?

Together with fellow-scientists from the Robert Koch Institute in Berlin and the Friedrich Loeffler Institute (Federal Research Institute for Animal Health) on the island of Riems, research scientists from the Fraunhofer Institute for Molecular Biology and Applied Ecology IME in Schmallenberg investigated these questions on behalf of the German Ministry for Environment, Nature Conservation and Nuclear Safety BMU. "We mixed soil samples with scrapie pathogens to find out how long the pathogens would survive," says Dr. Björn Seidel, who headed the investigations at IME. "Even after 29 months, in other words more than two years, we were still able to detect prions in the soil." But are these prions still infectious? "The soil actually seems to increase the infectiousness of the pathogens. The incubation period - the time it takes for the disease to break out - is exceedingly short even after the prions have persisted in the soil for 29 months. All of the animals that were given contaminated soil became sick within a very short time.

These results indicate that fresh incidences of scrapie among sheep are due to contaminated pastures," says Seidel in summary. The results of the study reveal that sheep may even become infected from the surface water, though the risk of infection is much lower in this case. There is no danger to humans, however: scrapie pathogens seem unable to affect them.

Another cause for concern is chronic wasting disease (CWD). Like BSE and scrapie, this is caused by prions, but it mainly affects deer. The numbers of infected animals in North America are rising steeply. How long do BSE and CWD prions survive in the ground? "To find this out, we urgently need to carry out further tests. The appropriate research applications have already been submitted," says Seidel.

http://www.eurekalert.org/pub_releases/2008-08/f-rp081108.php#

snip... full text ;

http://bse-atypical.blogspot.com/2008/11/resistance-of-bovine-spongiform.html

Friday, August 01, 2008 Excretion of transmissible spongiform encephalopathy infectivity in urine

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/excretion-of-transmissible-spongiform.html

Monday, May 12, 2008 Fecal transmission of AA amyloidosis in the cheetah contributes to high incidence of disease

http://betaamyloidcjd.blogspot.com/2008/05/fecal-transmission-of-aa-amyloidosis-in.html

Monday, January 05, 2009 CWD, GAME FARMS, BAITING, AND POLITICS

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html

Monday, September 1, 2008

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1] September 1, 2008

http://foiamadsheepmadrivervalley.blogspot.com/

http://foiamadsheepmadrivervalley.blogspot.com/2007/11/declaration-of-extraordinary-emergency.html

TSS

Thursday, January 15, 2009
Pathogenic prion protein is degraded by a manganese oxide mineral found in soils

http://chronic-wasting-disease.blogspot.com/2009/01/pathogenic-prion-protein-is-degraded-by.html

---------- https://lists.aegee.org/cjd-l.html ----------

CWD Update 93 December 29, 2008

flounder — Wed, 07 Jan 2009 10:03:40 GMT

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

CWD Update 93 December 29, 2008

State and Provincial Updates

South Dakota

The following was provided by Steve Griffin, South Dakota Game, Fish, and Parks:

In the South Dakota CWD Surveillance period of July 1, 2008 to December 3, 2008, a total of 1,470 samples have been collected for CWD surveillance. Breakdown of sampling is as follows: 292 elk sampled--277 results returned as NOT Positive--8 results pending (7 POSITIVE ELK FOUND) 319 mule deer sampled--184 results returned as NOT Positive--130 results pending (5 POSITIVE MD FOUND) 859 white-tailed deer--487 results returned as NOT Positive--368 results pending (4 POSITIVE WT FOUND)

Below is a listing of the Positive cervids that have been found in South Dakota during the surveillance period of July 1, 2008 to December 3, 2008.

1. Elk female from Wind Cave National Park in Custer County. (Sick/Surveillance)

2. Elk female from Unit H3B in Custer County. (Hunter Harvest)

3. Elk male from Unit H3A in Custer County. (Hunter Harvest)

4. Elk female from Wind Cave National Park in Custer County. (Sick/Surveillance)

5. Elk male from Unit H3A in Custer County. (Hunter Harvest)

6. White-tailed female from Unit BD4 in CusterCounty. (Sick/Surveillance)

7. Elk female from Unit H3C in Fall River County. (Hunter Harvest)

8. Elk female from Wind Cave National Park in Custer County. (Sick Surveillance)

9. White-tailed female from Unit 27A in Custer County. (Hunter Harvest

10. Mule deer male from Unit 27B in Fall River County. (Hunter Harvest)

11. Mule deer female from Unit 21B in Custer County. (Hunter Harvest)

12. White-tailed female from Unit 27B in Fall River County. (Hunter Harvest)

13. White-tailed male from Unit BD3 in Pennington County. (Sick/Surveillance)

14. Mule deer male from Unit 27B in Fall River County. (Hunter Harvest)

15. Mule deer female from Unit 27B in Fall River County. (Hunter Harvest)

16. Mule deer female from Unit 21B in Custer County. (Hunter Harvest)

In Summary:

South Dakota is reporting a total of 16 positive cervids (7 elk, 9 deer) in the testing period of July 1, 2008 to December 3, 2008. To date, South Dakota has found 93 cases of CWD (63 deer and 30 elk) in free ranging deer and elk since testing began in 1997. Wind Cave National Park accounts for 23 of these animals (15 elk, 8 deer). Three elk and 1 deer have been found in Custer State Park. A total of 18,873 wild deer and elk have been tested for CWD since 1997.

West Virginia:

The following was excerpted from a press release issued by the West Virginia DNR on December 22, 2008:

Five Additional Deer Test Positive for Chronic Wasting Disease in Hampshire County, West Virginia

Preliminary test results indicate the Chronic Wasting Disease (CWD) agent was present in five hunter-harvested deer collected in Hampshire County during the 2008 deer firearms hunting season.

"As part of our agency's ongoing and intensive CWD monitoring effort, samples were collected from 1,355 hunter-harvested deer brought to game checking stations in Hampshire County and one station near the southern Hampshire County line in Hardy County," noted Frank Jezioro, director for the West Virginia Division of Natural Resources (DNR).

The five CWD positive deer included one 4.5 year-old doe, two 2.5 year-old bucks, one 4.5 yearold buck and one 1.5 year-old buck. All five of the latest positive deer were harvested within the Hampshire County CWD Containment Area (i.e., that portion of Hampshire County located North of U.S. Route 50). However, the CWD agent previously has been detected outside the containment area but still within Hampshire County. The area in Hampshire County appears to continue to expand as one of the most recent infected deer was approximately five miles northeast of any previous known infected deer location.

CWD has now been detected in a total of 37 deer in Hampshire County (i.e., two road-killed deer - one in 2005 and one in 2008, four deer collected by the DNR in 2005, five deer collected by the DNR in 2006, one hunter-harvest deer taken during the 2006 deer season, three deer collected by the DNR in 2007, six hunter-harvested deer taken during the 2007 deer season, 11 deer collected by the DNR in 2008, and five hunter-harvested deer taken during the 2008 deer season). The DNR will continue to update management actions designed to control the spread of this disease, prevent further introduction of the disease, and possibly eliminate the disease from the state as information from deer testing within West Virginia is gathered and scientists across the country provide more information on how to combat CWD in white-tailed deer.

The entire press release can be viewed at:

http://www.wvdnr.gov/2008news/08news202.shtm.

Wisconsin:

CWD was detected in October 2008 on a captive cervid operation in Portage County (CWD Update 92). The facility was subsequently depopulated and an additional deer from the facility tested positive. A press release regarding this facility was issued on 12/17/208 by the Wisconsin Department of Agriculture, Trade and Consumer Protection and can be viewed at this link:

http://www.datcp.state.wi.us/press_release/result.jsp?prid=2259.

CWD was detected in another captive cervid operation in Wisconsin in December 2008. This facility is in Jefferson County and is the second facility with CWD detected in the state this year. A press release regarding this facility was issued on 12/19/208 by the Wisconsin Department of Agriculture, Trade and Consumer Protection and can be viewed at this link:

http://www.datcp.state.wi.us/press_release/result.jsp?prid=2262.

Wyoming

In October, 2008, Wyoming Game & Fish Department announced that a three-year-old female moose from far western Wyoming (Star Valley) tested positive for Chronic Wasting Disease. The press release regarding this detection can be viewed at:

http://gf.state.wy.us/services/news/pressreleases/08/10/17/081017_1.asp.

CWD has been detected in several new hunt areas in Wyoming this year: - Deer Hunt Area 27 (Southwest of Lake DeSmet) - Deer Hunt Area 2 (north of Sundance) - Elk Hunt Area 117 (near Sundance), - Elk Hunt Area 19 (South of Casper). Links to press releases concerning these new areas can be viewed at:

http://gf.state.wy.us/services/education/cwd/index.asp.

Meeting Announcement (reminder) The Third International CWD Symposium will be held July 22-24, 2009 in Park City, Utah. Information regarding the symposium, including registration, lodging, and the first Call for Papers, can be viewed at

http://www.regonline.com/cwd_symposium.

The deadline for abstract submission is February 20, 2009. For additional information contact Leslie McFarlane, Utah Division of Wildlife Resources, at

lesliemcfarlane@utah.gov.

Recent Publications The following article was published last week by PLOS One. The article documents a mule deer population with high CWD prevalence, high risk of CWD infection, and dramatically lowered (disease-associated) survival. The entire article is available at

http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0004019.

I urge all to read this article as it is the first publication I am aware of that links CWD with population decline: "Our findings provide compelling evidence that prion epidemics can affect mule deer population dynamics locally.."

Lions and Prions and Deer Demise

Michael W. Miller, Heather M. Swanson, Lisa L. Wolfe, Fred G. Quartarone, Sherri L. Huwer, Charles H. Southwick and Paul M. Lukacs PLoS ONE 3(12): e4019. doi:10.1371/journal.pone.0004019.

Abstract

Background: Contagious prion diseases - scrapie of sheep and chronic wasting disease of several species in the deer family - give rise to epidemics that seem capable of compromising host population viability. Despite this prospect, the ecological consequences of prion disease epidemics in natural populations have received little consideration. Methodology/Principal Findings: Using a cohort study design, we found that prion infection dramatically lowered survival of free-ranging adult (>2-year-old) mule deer (Odocoileus hemionus): estimated average life expectancy was 5.2 additional years for uninfected deer but only 1.6 additional years for infected deer. Prion infection also increased nearly fourfold the rate of mountain lions (Puma concolor) preying on deer, suggesting that epidemics may alter predator-prey dynamics by facilitating hunting success. Despite selective predation, about one fourth of the adult deer we sampled were infected. High prevalence and low survival of infected deer provided a plausible explanation for the marked decline in this deer population since the 1980s. Conclusion: Remarkably high infection rates sustained in the face of intense predation show that even seemingly complete ecosystems may offer little resistance to the spread and persistence of contagious prion diseases. Moreover, the depression of infected populations may lead to local imbalances in food webs and nutrient cycling in ecosystems in which deer are important herbivores.

http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0004019.

http://wildlifedisease.nbii.gov/documents/CWD%20Updates/update%2093.pdf

-------- Original Message --------

Subject: STUDY PLAN - Susceptibility of mountain lions to chronic wasting disease

Date: Tue, 5 Apr 2005 09:25:25 -0500

From: "Terry S. Singeltary Sr." flounder

Reply-To: Bovine Spongiform Encephalopathy <BSE-L@KALIV.UNI-KARLSRUHE.DE> To: BSE-L@KALIV.UNI-KARLSRUHE.DE

##################### Bovine Spongiform Encephalopathy #####################

Describe mechanisms of CWD transmission between infected and ...

snip...

Selective Predation by Mountain Lions on CWD-Infected Mule Deer In addition to direct effects on the habitats used by the natural cervid hosts of CWD, urbanization could affect the ecology of systems where CWD may be introduced or has become established. Because land use patterns may alter the abundance and activity of large predators (e.g., mountain lions), we have been studying the potential role of selective predation in CWD ecology. Our specific objectives are to:

1. test for evidence of selective predation by mountain lions on CWD-infected mule deer;

2. collect data to help assess the broader ecological question of whether mountain lions selectively prey on debilitated or compromised animals rather than healthy ones;

3. continue refining and assessing the adequacy of field sampling techniques for studying selective predation on CWD-infected mule deer; and

4. evaluate and compare the performance of Lotek Wireless GPS4000 and GPS4400 collars and Tevevilt GPS-Simplex collars in a study of selective predation by mountain lions under field conditions.

To test for evidence of selective predation, we will compare prevalence of CWD among puma-killed mule deer to prevalence among mule deer harvested or randomly culled by humans within home ranges of collared mountain lions. A total of eight adult mountain lions have been collared, resulting in 39 collared cat months between 2001 and present. Sampling of predator-killed deer is ongoing. ...

snip...

http://www.nrel.colostate.edu/projects/cwd/papers/CWD_2004_final.pdf

STUDY PLAN

Susceptibility of mountain lions to chronic wasting disease

M. W. Miller, L. L. Wolfe, and T. R. Davis

Background

Chronic wasting disease (CWD) naturally infects free-ranging deer (Odocoileus spp.) and elk (Cervus elaphus nelsoni) in northeastern Colorado and adjacent jurisdictions (Miller et al. 2000). Because CWD has the potential to destabilize affected cervid populations (Miller et al. 2000, Gross and Miller 2001), management efforts have been initiated to achieve goals of containing CWD and reducing its prevalence (Colorado Wildlife Commission 2001). Modeling studies have indicated that selective population control on affected populations may be most effective in controlling CWD (Gross and Miller 2001). It follows that processes fostering selective removal of affected individuals, like test-and-slaughter or predation, should be carefully evaluated in the context of CWD management.

Although the host range of CWD appears to be limited to deer and elk, several predatory species (including humans) may be exposed to prpCWD via consumption of infected carcasses. Because mountain lions (Felis concolor) are the most consistent predator of mule deer (0. hemionus) in the foothills of northeastern Colorado and southeastern Wyoming where CWD is most prevalent, they are undoubtedly exposed to CWD in areas where it occurs. To date, spongiform encephalopathy has not been detected in local mountain lion populations (M. W. Miller, unpubl. data). However, more formal studies of exposure rates (Krurnm and Miller 2002) and susceptibility of mountain lions to CWD are needed to clarify the role that mountain lions may play in helping manage CWD. Moreover, because mountain lions (Willoughby et al. 1992) and other wild felids (Kirkwood and Cunningham 1999) showed susceptibility to bovine spongiform encephalopathy (BSE) similar to that of humans (Will et al. 1996), understanding mountain lion susceptibility to CWD may provide insights into potential human susceptibility to CWD as well. Here, we describe plans for a study to evaluate natural susceptibility of captive mountain lions to CWD under conditions of prolonged exposure via consumption of infected mule deer carcasses.

Objectives

Our specific objective is to evaluate the potential susceptibility of mountain lions to CWD using a natural exposure route under controlled laboratory conditions.

Study Design

Three young (-4-6-week-old) mountain lions originating from near Chugwater, Wyoming were obtained opportunistically from the Wyoming Game and Fish Department in October 2001; these cubs were slated for euthanasia because no suitable rehabilitation destination was available to provide for their care. All 3 cubs have been maintained together at the CDOW Foothills Wildlife Research Facility in indoor or outdoor pens since that time. To date, their diet has consisted mainly of a commercial horse meat product (Dallas Crown, Inc., Kaufman, Texas) and healthy mule deer, supplemented occasionally with cottontail rabbits and house mice. In December 2001, we began operant conditioning training with all 3 cubs both to facilitate long-term tractability and veterinary care, and to provide behavioral enrichment; individual responses to this training also will serve as a baseline for evaluating behavioral changes that could be early clinical signs of CWD. In addition, the 2 male cubs were castrated and the female was spayed to further facilitate long-term tractability and to minimize potential for aggression, both intraspecific and toward caretakers. Other details of husbandry and care are described in FWRF Standard Operating Procedures.

Upon approval of this study plan, we will begin feeding all 3 cubs portioned carcasses from CWD-infected mule deer, such that at least 20% of their collective annual diet will be comprised of tissues from test-positive deer. This exposure level is about 33% higher than the highest mean CWD prevalence observed in northeastern Colorado (15% in GMU 9; Miller et al. 2000), thereby representing a substantial but not overwhelming treatment. Infected deer carcasses will be obtained opportunistically from captive and free-ranging sources in conjunction with ongoing research, surveillance, and management programs. All suspect carcasses will be confirmed via immunohistochemistry (IHC) or immunodotblot of brain and/or lymphoid tissues (Miller et al. 2000, Miller & Williams 2002, M. W. Miller unpubl. data). Carcasses will be prepared to specifically preserve central nervous system, gut, lymphoid, and muscle tissues, as well as other organ tissues, using an established protocol (Appendix A). Respective portions of each carcass will be identified and stored frozen until used. We will maintain a daily log of specific carcass portions fed and consumed, and will remove and dispose of uneaten portions at regular intervals.

Our study is planned to be descriptive, and consequently no experimental control will be maintained because spongiform encephalopathy does not appear to occur naturally in mountain lions (Williams et al. 2000). Instead, each lion will serve as its own control. We will monitor changes in body condition and behavior as indicators of clinical CWD in subject animals (Willoughby et al. 1992). Each lion will be weighed monthly and its responses to a series of routine training exercises scored subjectively. Persistent weight loss and/or other signs of disease will be evaluated, and treated as needed, by an attending veterinarian (Wolfe, Miller). If health problems become progressive and a lion fails to respond to treatment, that individual will be euthanized and subjected to a complete necropsy and diagnostic evaluation to rule out spongiform encephalopathy as the cause of illness. Similarly, any lion that dies will be subjected to complete necropsy and diagnostic evaluation. All 3 lions will be maintained for the duration of their lives under the foregoing experimental conditions.

We will document the extent of CWD exposure to CWD-infected mule deer, and will summarize onset, signs, and pathology of CWD in mountain lions in the unlikely event that illness occurs. Results will be described, but no analyses are planned.

Annual budget

Personal services $ 10,500 Operating $ 10,800

Literature Cited

Gross, J. E., and M. W. Miller. 2001. Chronic wasting disease in mule deer: A model of disease dynamics, control options, and population consequences. J. Wildl. Manage., in press.

Kirkwood, J. K., and A. A. Cunningham. 1999. Scrapie-like spongiform encephalopathies (prion diseases) in nondomesticated species. In Zoo and wild animal medicine, 3rd edition. M. E. Fowler and R. E. Miller, eds., W. B. Saunders, Philadelphia, Pennsylvania, pp. 662-668.

Krumm, C. T., and M. W. Miller. 2002. Study plan: Selective predation by mountain lions on chronic-wasting disease-infected mule deer. In prep.

Miller, M. W., E. S. Williams, C. W. McCarty, T. R. Spraker, T. J. Kreeger, C. T. Larsen, and E. T. Thorne. 2000. Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. Journal of Wildlife Diseases 38:676-690.

Will, R. G., J. W. Ironside, M. Zeidler, S. N. Cousens, K. Estibeiro, A. Alperovitch, S. Poser, M. Pocchiari, A. Hoffman, and P. G. Smith. 1996. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347: 921-925.

Willoughby, K., D. F. Kelly, D. G. Lyon, and G. A. H. Wells. 1992. Spongiform encephalopathy in a captive puma (Felis concolor). Veterinary Record 13 l:431-434.

Williams, E. S., J. A. Kirkwood, and M. W. Miller. 2000. Transmissible spongiform encephalopathies. In Infectious diseases of wild mammals, 3rd edition. E. S. Williams and I. K. Barker, eds. Iowa State University press, Ames, Iowa, pp. 292-301.

Appendix A

Carcass Processing for Mountain Lions

I. Carcass labeling and CWD Tissue Sampling:

1) Carcasses intended for the lions should be labeled to avoid mixing them up with those intended for necropsy. Lion carcasses must be labeled "Lions", dated, and assigned a CWD case number when they come in (please use wire twist labels and a permanent marker). If the animal is a known positive or negative CWD animal, please indicate this also on the tag. Whoever removes the head from the carcass for CWD sampling is also responsible for bagging and labeling it with all of the above information.

2) If the labeled carcass will be processed within a few days it can be left in the cooler, otherwise it should go into the freezer to prevent tissue decomposition. Carcasses will need to be pulled out of the freezer to thaw two days in advance of processing and should be placed in the cooler and/or in the necropsy lab.

3) Remove the head and take CWD tissue samples according to WHL protocol. WHL personnel may have already done this.

4) Most of the carcasses you process will be CWD "unknowns" as test results are usually necessary to indicate if the carcass is CWD positive or negative. All heads must be saved until CWD test results are in.

5) Process the carcass and store the tissues according to instructions below.

6) Enter the case number and all necessary information into the blue Lion Carcass Notebook.

II. Processing the Carcass;

Processing known negatives (fawns), or "green" carcasses (treat these as negatives);

1) Skin the carcass and toss out the hide according to WHL disposal protocol.

2) The large bones should be removed and saved in a labeled bag (with or without the hide, and it is good to leave large chunks of meat attached).

3) Cut up meat from each front and hindquarter into approximately fist size chunks, and mix in chopped pieces of heart, liver, and kidney. Store in blue tubs or labeled ziplock bags, and fresh freeze. These tissues will be fed to the lions to supplement our commercial feline diet.

Additional tissues to collect for suspect CWD positive animals:

4) Slice off 2-3 sections of the neck with skin intact and place into a labeled (twist tie) garbage bag with the processed head and sections of spine. If WHL personnel processed the head you will need to find this bag in the freezer (CWD case number on the carcass should match the one on the bagged head), and add the spine and neck sections to the same bag. These tissues will be fed to the lions as part of the study protocol.

5) Open the abdomen and place the following tissues into separately labeled whirlpacks, and store these in the blue tub labeled "Unknown CWD Organ Tissues", and fresh freeze.

a.) Liver

b.) Lower 10 inches of the colon

c.) Kidney

d.) Urine (if the carcass is fresh) - store in a labeled plastic centrifuge tube.

These tissues are set aside for laboratory analysis, and will not be fed to the lions.

6) Remove the digestive tract and store in a labeled twist tie garbage bag and fresh freeze. The digestive tract will be fed to the lions.

Additional tissues to collect for known positive CWD animals:

7) Fetuses should be removed, saved in labeled bags, and fresh frozen.

8) Save several cotyledons from the uterus (or the entire repro. tract if cotyledons are not obvious). Fresh freeze several in labeled whirlpacks, and store one in a labeled jar with formalin.

9) Ticks and bots should also be fresh frozen, and stored in labeled whirlbacks.

These tissues are also set aside for laboratory analysis and will not be fed to the lions.

10) Please follow instructions below for labeling and storing these tissues.

11) Please clean up your work area, and dispose of waste according to WHL protocols (attached).

III. Tissue Labeling and Tracking Samples:

1) All storage containers and bags must be clearly labeled with:

a. LIONS, or "L"

b. The date the carcass came in

c. The CWD case number

d. The tissue type (meat, bones, head/neckl/spine, organs, gut, fetus, cotyledons, tickshots)

e. CWD +,-, or unknown.

Known Positives;

2) Known CWD positive tissues are color coded green and stored on the shelves in the NW comer of the freezer.

Known Negatives:

3) Known negative tissues are color coded blue and stored on the shelves in the SW comer of the freezer (shelves to the left of the door).

Unknowns:

4) Unknown head/neck/spine sections and gut piles are stored in the red Rubbermaid bin and are not color coded until CWD test results are obtained.

5) Unknown (but suspected to be positive) organs are stored in the blue tub on the SW shelf and the tub is labeled "Unknown CWD Organ Tissues".

6) When CWD test results come in, WHL personnel will indicate if each case number is positive or negative in the blue lion carcass notebook.

7) Please go through unknown CWD organ tissues (blue tub), head/neck/spine, and gut contents (red bin) once a week to color code and separate out.

a. Negative head/neck/spine sections, gut piles and organ tissues should be tossed out (see WHL lab protocol for disposal). However we should save a few negative organ tissues for comparative lab work.

b. Positive head/neck/spine sections, and gut piles should be moved to the CWD positive section of the freezer (NW comer). Positive organ tissues should be moved to the green tub labeled "Positive CWD Organ Tissues" on the NW shelves.

IV. Feeding the lion:

8) All meat and large bones should be saved and fed out regularly. Lions should also receive one gut pile/week. Meat from known CWD positive animals is stored in green tubs, meat from known CWD negative animals is stored in blue tubs. "Unknown" meat is stored in ziplock bags.

9) Meat, bones and gut can be fed out even if they are unknowns, however it is necessary to track these tissues as part of the study protocol. Please fill out the following information in the blue lion observation notebook (located in the lion shed) prior to feeding out any processed tissues or carcasses:

a) tissue type

b) CWD case number

c) CWD +, -, or unknown at the time of feeding

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########

Thursday, December 25, 2008

Lions and Prions and Deer Demise

http://chronic-wasting-disease.blogspot.com/2008/12/lions-and-prions-and-deer-demise.html

Wednesday, December 17, 2008

ONE-THIRD OF BOULDER'S DEER INFECTED WITH CWD

http://chronic-wasting-disease.blogspot.com/2008/12/one-third-of-boulders-deer-infected.html

Thursday, December 25, 2008

Elk meat recalled due to CWD Boulder County Health Department and Colorado Department of Public Health and Environment

http://chronic-wasting-disease.blogspot.com/2008/12/elk-meat-recalled-due-to-cwd-boulder.html

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

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

also,

A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most

serious because of rapid horizontal spread and higher prevalence than BSE in

UK, up to 15% in some populations. Also may be a risk to humans - evidence

that it is not dangerous to humans is thin.

http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summar

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,? Michael W. Miller,? Pierluigi Gambetti,§ and Lawrence B. Schonberger*

*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; ?University of Wyoming, Laramie, Wyoming, USA; ?Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from:http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

--------------------------------------------------------------------------------

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.

snip...full text ;

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

Volume 12, Number 10-October 2006

Research

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Samantha MaWhinney,* W. John Pape,? Jeri E. Forster,* C. Alan Anderson,?§ Patrick Bosque,?¶ and Michael W. Miller#

*University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; ?Colorado Department of Public Health and Environment, Denver, Colorado, USA; ?University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Suggested citation for this article

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40-1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73-1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

snip... full text ;

http://0-www.cdc.gov.mill1.sjlibrary.org/ncidod/EID/vol12no10/06-0019.htm

full text ;

http://chronic-wasting-disease.blogspot.com/2006_12_01_archive.html

CHRONIC WASTING DISEASE BLOG

http://chronic-wasting-disease.blogspot.com/

http://stanford.wellsphere.com/healing---recovery-article/a-prion-disease-of-cervids:-chronic-wasting-disease-2008/13819

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 ;

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

Sunday, November 23, 2008

PRION October 8th - 10th 2008 Book of Abstracts

http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html

Saturday, September 06, 2008 Chronic wasting disease in a Wisconsin white-tailed deer farm 79% INFECTION RATE Contents: September 1 2008, Volume 20, Issue 5

snip...see full text ;

http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html

Wednesday, December 17, 2008 White-tailed Deer in Portage County Tests Positive for CWD

http://chronic-wasting-disease.blogspot.com/2008/12/white-tailed-deer-in-portage-county.html

Monday, December 22, 2008

CWD DETECTED IN ELK HUNT AREA 117 SOUTH OF SUNDANCE WYOMING

http://chronic-wasting-disease.blogspot.com/2008/12/cwd-detected-in-elk-hunt-area-117-south.html

Monday, January 05, 2009

CWD, GAME FARMS, BAITING, AND POLITICS

http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html

The prion strain phenomenon: Molecular basis and unprecedented features

http://bse-atypical.blogspot.com/2008/12/prion-strain-phenomenon-molecular-basis.html

Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

Thursday, November 13, 2008

SIXTEENTH ANNUAL REPORT 2007 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK

http://creutzfeldt-jakob-disease.blogspot.com/2008/11/sixteenth-annual-report-2007.html

Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

Tuesday, January 06, 2009
CWD Update 93 December 29, 2008
http://chronic-wasting-disease.blogspot.com/2009/01/cwd-update-93-december-29-2008.html

-------------------- BSE-L@LISTS.AEGEE.ORG --------------------

North American Cervids Harbor Two Distinct CWD Strains

flounder — Tue, 16 Dec 2008 13:09:41 GMT

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 ;

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

Sunday, November 23, 2008

PRION October 8th - 10th 2008 Book of Abstracts

http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html

Biochim Biophys Acta. Author manuscript; available in PMC 2008 December 9. Published in final edited form as: Biochim Biophys Acta. 2007 June; 1772(6): 681-691. Published online 2006 December 15. doi: 10.1016/j.bbadis.2006.12.006. PMCID: PMC2597801 NIHMSID: NIHMS25810

The prion strain phenomenon: Molecular basis and unprecedented features

Rodrigo Morales,1,2 Karim Abid,1 and Claudio Soto1# 1 Protein Misfolding Disorders Laboratory, George and Cynthia Mitchell Center for Neurodegenerative diseases, Departments of Neurology, Neuroscience & Cell Biology and Biochemistry & Molecular Biology, University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas, 77555-0646, USA 2 Facultad de Ciencias, Universidad de Chile, Santiago, Chile #To whom correspondence should be addressed at Email: clsoto@utmb.edu The publisher's final edited version of this article is available at Biochim Biophys Acta. See other articles in PMC that cite the published article.

Abstract

Prions are unconventional infectious agents responsible for transmissible spongiform encephalopathies. Compelling evidences indicate that prions are composed exclusively by a misfolded form of the prion protein (PrPSc) that replicates in the absence of nucleic acids. One of the most challenging problems for the prion hypothesis is the existence of different strains of the infectious agent. Prion strains have been characterized in most of the species. Biochemical characteristics of PrPSc used to identify each strain include glycosylation profile, electrophoretic mobility, protease resistance, and sedimentation. In vivo, prion strains can be differentiated by the clinical signs, incubation period after inoculation and the vacuolation lesion profiles in the brain of affected animals. Sources of prion strain diversity are the inherent conformational flexibility of the prion protein, the presence of PrP polymorphisms and inter-species transmissibility. The existence of the strain phenomenon is not only a scientific challenge, but it also represents a serious risk for public health. The dynamic nature and inter-relations between strains and the potential for the generation of a very large number of new prion strains is the perfect recipe for the emergence of extremely dangerous new infectious agents.

snip...

BSE has not only been transmitted to humans. The extensive use of cow-derived material for feeding other animals led to the generation of new diseases in exotic felines such as tiger and cheetah, non human primates, and domestic cats [52,57-60]. As it was mentioned before, the transmission of BSE into these different species could create many new prion strains, each one of them with particular biological and biochemical characteristics and thus a potentially new hazard for human health. Successful transmission of BSE in pigs has been described [61,62] and also in transgenic mice expressing pig PrP (PoPrP) [63]. Porcine derivates are widely consumed and the hypothetic case of "mad pigs" could increase the events of zoonotic transmission of prions to humans. Fortunately, transmission of BSE to pigs is possible only in very drastic conditions, not likely to be occurring naturally [62,63]. More frightening is perhaps the possibility that BSE has been passed into sheep and goats. Studies have already shown that this transmission is possible and actually relatively easy and worrisomely produces a disease clinically similar to scrapie [64]. The cattle origin of this new scrapie makes possible that the new strain may be transmissible to humans. Transmission experiments of BSE infected sheep brain homogenate into human transgenic animal models are currently ongoing in several laboratories. It is very important to note that all materials generated by transmission of BSE in experimental and natural cases show similar biochemical behavior compared to the original inoculum [65], suggesting that all these new generated infectious agents could potentially be hazardous for humans. The origin of BSE is still a mystery. Abundant evidence supports the hypothesis that BSE was produced by cattle feeding with scrapie derivated material [66,67], indicating that bovine PrPSc might be a "conformational intermediary" between ovine PrPSc and human PrPC.

There is currently no mean to predict which will be the conformation of a newly generated strain and how this new PrPSc conformation could affect other species. One interesting new prion disease is CWD, a disease affecting farm and wild species of cervids [68,69]. The origin of CWD and its potential to transmit to humans are currently unknown. This is worrisome, considering that CWD has became endemic in some parts of USA and the number of cases continues to increase [69]. It is presumed that a large number of hunters in the US have been in contact or consumed CWD-infected meat [70]. CWD transmissibility studies have been performed in many species in order to predict how this disease could be spread by consumption of CWD meat [71-73]. In these studies, a special attention has been done to scavenging animals [74], which are presumed to be exposed to high concentration of cervid prions, resulting in the putative generation of many new forms of TSEs. Fortunately negative results were obtained in one experiment done in raccoons infected with CWD [74]. Transmission of CWD to humans cannot be ruled out at present and a similar infective episode to BSE involving CWD could result in catastrophic events, spreading the disease in a very dangerous way through the human population. No clinical evidence linking CWD exposed humans and CJD patients have been found [70], but experimental inoculation of CWD prions into squirrel monkeys propagated the disease [71]. It is important to mention that the species barrier between humans and cervids appears to be greater than with cattle, as judged by experiments with transgenic mice models [75]. Finally, it is important to be aware about CWD transmissibility to other species in which a "conformational intermediary" could be formed, facilitating human infection.

SNIP...

VI. Unique features of prion strains

The biological and infectious characteristics of prions are dramatically different to the conventional infectious agents. These differences are manifested in the prion strains phenomenon in unique and unprecedented features, such as for example strain adaptation and memory, the coexistence and competition of prion strains, among others. In this section, some of these interesting phenomena will be briefly described.

Adaptation of Prion strains

Interspecies transmission of prions could result in the emergence of more than one variety of infectious material. All new collected infectious agents could present particular strain characteristics. That is the case of DY and HY prion strains generation [13,16]. When interspecies transmission of prions occurs, serial passages in the new host are needed in order to stabilize the characteristics of new generated infectious material. In the case of TME transmission in hamsters, at least four serial passages in the new species were required for stabilization [13]. The first passage was characterized by long incubation periods and a dominance of a 19 KDa fragment when newly obtained PrPSc was analyzed after PK digestion. In the three first passages, clinical symptoms were not characteristic of the hamster-adapted HY or DY TME strains. This phenotype was attributed to the combination effects of both strains replicating simultaneously. Thereafter, each of the strains was stabilized in some of the animals and once they are adapted and stabilized, they can be serially propagated in vivo and the characteristics are maintained. It is accepted that both strains present differential conversion kinetics in vitro, with DY being the slowest and HY the fastest [124]. For this reason, in order to select efficiently this prion strain, limit dilutions must be performed [13]. In that way, the most abundant and less convertible DY is favored against the less abundant but fastest HY strain.

Co-existence of prion strains

Related to the above, it has been shown that two or more prion strains can co-exist in natural cases of TSE. Co-existence of prion strains has been found in sporadic cases of CJD [113, 125]. Analyses of several sCJD tissue showed that different biochemical profiles of PrPSc could be found in different brain areas from the same patient [113]. Co-existence of prion strains was mainly observed in patient heterozygous for codon 129 [113]. As many as 50% of these patients present different types of PrPSc in their brains, whereas 9% of MM patients were positive for co-existence of strains. On the other hand, more than one PrPSc type was not observed in VV patients [113].

The biochemical and structural properties of the protein seem to be the major cause of this differential distribution. This observation may explain why sCJD is so heterogeneous in terms of clinical manifestation [34,126,127]. In a recent publication by Bishop et al. [107], vCJD infected transgenic mice expressing human PrPC, present changes in their PrPSc and vacuolation patterns in the brain according to their polymorphic classification for codon 129.

Competition of prion strains

In particular experimental conditions, some prion strains can extend their specific incubation period when co-infected with another strain. Long incubation period prions increase the incubation period of "faster" prions. This phenomenon of "competition of prion strains" has been observed in mice and hamster. In mice, competition between 22A and 22C strains was reported in 1975 by Dickinson et al. [128]. In this study, RIII mice (homozygous for sincs7 allele) were used. 22A and 22C showed long and short incubation period (550 and 230 days), respectively. When 22C strain was intraperitoneally inoculated 100, 200 and 300 days after intraperitoneal administration of the 22A agent, all three experimental groups resulted in Morales et al. Page 8 Biochim Biophys Acta. Author manuscript; available in PMC 2008 December 9. NIH-PA Author Manuscript NIH-PA Author Manuscript NIH-PA Author Manuscript incubation periods and lesion patterns matching 22A prions, suggesting that 22C prions were degraded or excreted, in animals previously infected by 22A. Similar results were obtained by Kimberlin and Walker in 1985 [129] using a different strain of sincs7 mice. These authors treated mice using 22A and 22C prion strain. Before inoculation, 22A was treated with different chemical and physical agents in order to see if the "competitor" or "blocking" characteristics of 22A were maintained. From all treatments, 12M urea was shown to almost abolish the blocking properties of 22A agent. This information suggests that infective properties of long incubation period agent are strictly necessary in order to increase the incubation period of faster prions.

In hamster, similar observations were reported using DY and HY [130]. DY prion strain was inoculated 30 and 60 days prior intraperitoneal inoculation of HY at three different doses. When incubation periods of HY inoculated control group were compared with the animals inoculated at 60 days with DY, significant differences in the incubation periods were found, especially when HY prions were administrated in a higher dose [130]. On the other hand no differences were observed in the case of intranerve inoculation, revealing that competition phenomenon occurs only when peripheral inoculation is performed. These results are surprising considering the fact that DY was reported not to be infectious when intraperitoneally inoculated in hamsters [130]. This data suggest that replication of DY is occurring in peripheral tissues but is not able to reach the central nervous system.

In general, the principal variables that need to be observed for a successful competition are the route of infection, the interval between injections and the particular strains and doses of agent used. Prolongation of incubation periods in TSE are therapeutically beneficial and several strategies are under development to reach this aim, including antibodies, beta-sheet breakers, and other chemical agents [131-133]. The experimental evidence described above suggests that prions could be potentially useful for this purpose. In order to prevent spread of prion disease in cattle or humans, prion strains with incubation periods longer than species' lifespan could be used to slowdown the replication of BSE or vCJD prions.

VII. Concluding Remarks

The existence of different strains of an infectious agent composed exclusively of a protein has been one of the most puzzling issues in the prion field. If is already difficult to understand how a protein can adopt two stable and different folded structures and that one of them can transform the other one into itself, it is unthinkable that the misfolded form can in turn adopt multiple conformations with distinct properties. Yet, compelling scientific evidence support the idea that PrP can adopt numerous folding patterns that can faithfully replicate and produce different diseases. The existence of the strain phenomenon is not only a scientific challenge, but it also represents a serious risk for public health. The dynamic nature and inter-relations between strains and the potential for the generation of many new prion strains depending on the polymorphisms and the crossing of species barrier is the perfect recipe for the emergence of extremely dangerous new infectious agents. Although, substantial progress has been made in understanding the prion strains phenomenon, there are many open questions that need urgent answers, including: what are the structural basis of prion strains?; how are the phenomena of strain adaptation and memory enciphered in the conformation of the prion agent?; to what species can a given prion strain be transmissible?; what other cellular factors control the origin and properties of prion strains?. ...SNIP...END

http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2597801&rendertype=abstract

http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=2597801&blobtype=pdf

Friday, December 12, 2008

The prion strain phenomenon: Molecular basis and unprecedented features

http://bse-atypical.blogspot.com/2008/12/prion-strain-phenomenon-molecular-basis.html

Sunday, November 30, 2008

Commentary: Crimes hurt essence of hunting

http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html

TSS

CWD and Deer Diseases

Species Barrier May Protect Macaques from Chronic Wasting Disease

U.S. study: deer infected with chronic wasting disease safe to eat

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

Monday, July 13, 2009

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

Infectious Prions in Pre-Clinical Deer and Transmission of Chronic Wasting Disease Solely by Environmental Exposure

CWD UPDATE Infection Studies in Two Species of Non-Human Primates and one Environmental reservoir infectivity study and evidence of two strains

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD) CDC WARNING 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have Chronic Wasting

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

Pathogenic prion protein is degraded by a manganese oxide mineral found in soils

CWD Update 93 December 29, 2008

North American Cervids Harbor Two Distinct CWD Strains

Sunday, November 30, 2008

Commentary: Crimes hurt essence of hunting http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html

Commentary: Crimes hurt essence of hunting

http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html