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1,446 Posts
Discussion Starter · #1 ·
Subject: Prion disease found lurking in deer muscle
Date: January 26, 2006 at 11:44 am PST

Prion disease found lurking in deer muscle
19:00 26 January 2006
NewScientist.com news service
Debora MacKenzie

The infectious prions that cause Chronic Wasting Disease, an infection similar to BSE that afflicts North American deer and elk have been found in the parts of the animals that people eat. No one knows if CWD can jump to humans, but if it does hunters in affected areas might be at risk.

CWD was first diagnosed as a spongiform encephalopathy in captive deer and elk in Colorado in the 1970s, and in wild deer and elk in the region in the 1980s. But in the 1990s it spread widely within the elk farming industry, jumped to wild deer, and now affects two provinces of Canada and 13 US states.

Like the related sheep disease scrapie – though unlike BSE – CWD spreads from animal to animal, says Glenn Telling of the University of Kentucky at Lexington, US. Deer housed with infected animals, or fed infected brain experimentally, contract the disease.

Because of this there are fears that the CWD prion might be distributed widely in the deer’s tissues – as scrapie is in sheep. Efforts to find the infectious prion in the muscle of infected animals, by seeing whether antibodies to the prion could find any and bind on, have previously failed.

But Telling’s lab has now shown that diseased prions can reside in muscle of deer infected with CWD, by using transgenic mice.

Transgenic mice
The team replaced the gene for the normal mouse version of the prion protein with the normal gene from deer, so the mice made the normal, healthy deer protein. They then injected the mouse brains with tissue from infected deer. Twelve to 18 months later, the mice developed encephalopathy.

Tissues from both the infected deers' brains and thigh muscle caused disease. Muscle took slightly longer to cause disease than brain tissue, showing it had slightly less prion.

“We don’t know that it is transmitted in the wild by animals eating muscle from infected animals,” cautions Telling. “We now have to see where else the prion might be,” including saliva and even excrement, using more transgenic mice.

Brain warnings
“Because we tested deer that were already ill,” he told New Scientist, “we don’t know what the distribution of prion is in animals that are still incubating the disease.” Hunters have been warned by wildlife agencies not to kill and eat obviously ill animals, but an animal not yet showing signs of the disease might still carry the abnormal prion, albeit less of it.

It is also unknown whether people can catch encephalopathy by eating CWD-infected meat, as they can from eating BSE-tainted meat. Anecdotal reports that hunters develop the human prion disease CJD in unusual numbers have never been confirmed. State officials have issued warnings to hunters not to eat brain or spinal cord – the tissues most affected.

“If I were a hunter I would be cautious about eating deer in areas affected,” says Telling. But he notes that not much testing of wildlife has been done, and it is not clear how prevalent the infection is.

Journal reference: Science (DOI: 10.1126/science.1122864)



1,446 Posts
Discussion Starter · #2 ·
Subject: Prions in Skeletal Muscles of Deer with Chronic Wasting Disease [SCIENCE FULL TEXT]
Date: January 26, 2006 at 12:23 pm PST

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease

Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§

1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA.

*These authors contributed equally to this work.

†Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA.

‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.

§To whom correspondence should be addressed: E-mail: [email protected]

Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD.

To test whether skeletal muscle of diseased cervids contained prion infectivity, Tg(CerPrP)1536 mice (2) expressing cervid prion protein (CerPrP), were inoculated intracerebrally with extracts prepared from the semitendinosus/semimembranosus muscle group of CWD-affected mule deer or from CWD-negative deer. The availability of CNS materials also afforded direct comparisons of prion infectivity in skeletal muscle and brain. All skeletal muscle extracts from CWD-affected deer induced progressive neurological dysfunction in Tg(CerPrP)1536 mice with mean incubation times ranging between 360 and ~490 d, whereas the incubation times of prions from the CNS ranged from ~230 to 280 d (Table 1). For each inoculation group, the diagnosis of prion disease was confirmed by the presence of PrPSc in the brains of multiple infected Tg(CerPrP)1536 mice (see supporting online material for examples). In contrast, skeletal muscle and brain material from CWD-negative deer failed to induce disease in Tg(CerPrP)1536 mice (Table 1) and PrPSc was not detected in the brains of sacrificed asymptomatic mice as late as 523 d after inoculation (supporting online material).

Our results show that skeletal muscle as well as CNS tissue of deer with CWD contains infectious prions. Similar analyses of skeletal muscle BSE-affected cattle did not reveal high levels of prion infectivity (3). It will be important to assess the cellular location of PrPSc in muscle. Notably, while PrPSc has been detected in muscles of scrapie-affected sheep (4), previous studies failed to detect PrPSc by immunohistochemical analysis of skeletal muscle from deer with natural or experimental CWD (5, 6). Since the time of disease onset is inversely proportional to prion dose (7), the longer incubation times of prions from skeletal muscle extracts compared to matched brain samples indicated that prion titers were lower in muscle than in CNS where infectivity titers are known to reach high levels. Although possible effects of CWD strains or strain mixtures on these incubation times cannot be excluded, the variable 360 to ~490 d incubation times suggested a range of prion titers in skeletal muscles of CWD-affected deer. Muscle prion titers at the high end of the range produced the fastest incubation times that were ~30% longer than the incubation times of prions from the CNS of the same animal. Since all mice in each inoculation group developed disease, prion titers in muscle samples producing the longest incubation times were higher than the end point of the bioassay, defined as the infectious dose at which half the inoculated mice develop disease. Studies are in progress to accurately assess prion titers.

While the risk of exposure to CWD infectivity following consumption of prions in muscle is mitigated by relatively inefficient prion transmission via the oral route (8), these

results show that semitendinosus/semimembranosus muscle, which is likely to be consumed by humans, is a significant source of prion infectivity. Humans consuming or handling meat from CWD-infected deer are therefore at risk to prion exposure.

References and Notes

1. P. J. Bosque et al., Proc. Natl. Acad. Sci. U.S.A. 99, 3812 (2002).

2. S. R. Browning et al., J. Virol. 78, 13345 (2004).

3. A. Buschmann, M. H. Groschup, J. Infect. Dis. 192, 934 (2005).

4. O. Andreoletti et al., Nat. Med. 10, 591 (2004).

5. T. R. Spraker et al., Vet. Pathol. 39, 110 (2002).

6. A. N. Hamir, J. M. Miller, R. C. Cutlip, Vet. Pathol. 41, 78 (2004).

7. S. B. Prusiner et al., Biochemistry 21, 4883 (1980).

8. M. Prinz et al., Am. J. Pathol. 162, 1103 (2003).

9. This work was supported by grants from the U.S. Public Health Service 2RO1 NS040334-04 from the National Institute of Neurological Disorders and Stroke and N01-AI-25491 from the National Institute of Allergy and Infectious Diseases.

Supporting Online Material


Materials and Methods

Fig. S1

21 November 2005; accepted 13 January 2006 Published online 26 January 2006; 10.1126/science.1122864 Include this information when citing this paper.

Table 1. Incubation times following inoculation of Tg(CerPrP)1536 mice with prions from skeletal muscle and brain samples of CWD-affected deer.

Inocula Incubation time, mean d ± SEM (n/n0)*

Skeletal muscle Brain

CWD-affected deer

H92 360 ± 2 d (6/6) 283 ± 7 d (6/6)

33968 367 ± 9 d (8/8) 278 ± 11 d (6/6)

5941 427 ± 18 d (7/7)

D10 483 ± 8 d (8/8) 231 ± 17 d (7/7)

D08 492 ± 4 d (7/7)

Averages 426 d 264 d

Non-diseased deer

FPS 6.98 >523 d (0/6)

FPS 9.98 >454 d (0/7) >454 d (0/6)

None >490 d (0/6)

PBS >589 d (0/5)

*The number of mice developing prion disease divided by the original number of inoculated mice is shown in parentheses. Mice dying of intercurrent illnesses were excluded.



Supporting Online Material for

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease

Rachel C. Angers, Shawn R. Browning, Tanya S. Seward, Christina J. Sigurdson,

Michael W. Miller, Edward A. Hoover, Glenn C. Telling§

§To whom correspondence should be addressed: E-mail: [email protected]

Published 26 January 2006 on Science Express

DOI: 10.1126/science.1122864

This PDF file includes:

Materials and Methods

Fig. S1

Supporting Online Materials

Materials and Methods

Homogenates of semitendinosus/semimembranosus muscle (10% w/v in phosphate

buffered saline) were prepared from five emaciated and somnolent mule deer, naturally

infected with CWD at the Colorado Division of Wildlife, Wildlife Research Center.

These deer were identified as D10, D08, 33968, H92, and 5941. CWD infection was

confirmed in all cases by the presence of histologic lesions in the brain including

spongiform degeneration of the perikaryon, the immunohistochemical detection of

disease-associated PrP in brain and tonsil, or by immunoblotting of protease-resistant,

disease associated PrP (CerPrPSc). Semitendinosus/semimembranosus muscle was also

obtained from two asymptomatic, mock inoculated deer, referred to as FPS 6.68 and 9.98,

that originated from a CWD non-endemic area and which were held indoors at Colorado

State University from ten days of age. These control deer were confirmed negative for

CWD by histopathological and immunohistochemical analysis of brain tissue at autopsy.

The utmost care was taken to avoid inclusion of obvious nervous tissue when muscle

biopsies were prepared and to ensure that contamination of skeletal muscle samples with

CNS tissue did not occur. Fresh, single-use instruments were used to collect each sample

biopsy and a central piece from each sample was prepared with fresh, disposable

instruments to further isolate muscle tissue for inoculum preparation. Brain samples for

transmission were prepared separately from muscle as additional insurance against cross



Groups of anesthetized Tg(CerPrP)1536 mice were inoculated intracerebrally with 30 µl

of 1 % skeletal muscle or brain extracts prepared in phosphate buffered saline (PBS).

Inoculated Tg(CerPrP) mice were diagnosed with prion disease following the progressive

development of at least three neurologic symptoms including truncal ataxia, ‘plastic’ tail,

loss of extensor reflex, difficultly righting, and slowed movement. The time from

inoculation to the onset of clinical signs is referred to as the incubation time.

For PrP analysis in brain extracts of Tg(CerPrP)1536 mice, 10 % homogenates prepared

in PBS were either untreated (-) or treated (+) with 40 µg/ml proteinase K (PK) for one

hour at 37oC in the presence of 2% sarkosyl. Proteins were separated by sodium dodecyl

sulfate polyacrylamide gel electrophoresis, analyzed by immunoblotting using anti PrP

monoclonal antibody 6H4 (Prionics AG, Switzerland), incubated with appropriate

secondary antibody, developed using ECL-plus detection (Amersham), and analyzed

using a FLA-5000 scanner (Fuji).


Fig. S1

PrP in brain extracts from representative Tg(CerPrP)1536 mice receiving muscle or CNS

tissue inocula from CWD-affected or CWD-negative deer. Extracts were either treated

(+) or untreated (-) with proteinase K (PK) as indicated. The positions of protein

molecular weight markers at 21.3, 28.7, 33.5 kDa (from bottom to top) are shown to the

left of the immunoblot.



price of poker goes up. ...TSS:bash: :bash: :bash:

4,056 Posts
So what is next???? Are we gonna end up shooting deer for the sport of it and then just wasting everything but the antlers. Seems like there are more and more diseases affecting deer every year.


495 Posts
If I understand things correctly, we know that prions exist in infected animals but do not know with any certainty whether prions are a cause or a symptom. We know that pens that have held infected cervids even after cleaning and after being empty for some time that their subsequent housing of healthy animals cause these animals to be later diagnosed with CWD. This suggests that the passage of time does not eliminate the causative agent. We know that in total TSE's have crossed species boundaries. We once believed that TSE, BSE, and CWD infections were localized to brain, head, or specific areas and we now know that virtually any muscle group can be infected. We know that symptoms are not always observable and this is particularly true in the early stages. In fact, we really don't have a clue as to how the various TSE's are transmitted beyond eating infected tissue as there is clear evidence that some have become infected by eating tissue from cattle with Mad Cow Disease? I guess I can only speak for myself but I only hunt for meat and killing just for the sport or for the antlers is somewhat against my moral code. If I need to shoot something that bad then for me it would be time to lock away the guns. Given all that we know or if my understandings are correct and given that I only hunt for meat, I would have to ask why would I or anyone in a potential CWD area even think about wanting to hunt? Wiping out the cervids in a locale can not equate to elimination of the disease so hunting and particularly hunting to the point of elimination seems fruitless as we don't know the causative agent and knowing that absence and the passage of time doesn't eliminate the disease.

If I understand things correctly then in my mind the sum of all of this is devastating news. If I have indeed captured some of the salient points regarding CWD/TSE's then why are we not hearing more about this in the news and why are we not seeing an outcry for higher expenditures for research? From the perspective of wildlife biologists considering credible research, is there any evidence that the Wisconsin deer slaughter has eliminated the disease or stopped the spread locally? What has been learned with the outbreaks and controls applied so far in Wisconsin? At this time our state plans regarding CWD have to be dynamic and if so what news can we look for from the state? Finally, considering the recent Mad Cow Disease concerns, has there been any linkages with or between the cattle and cervids as aren't we generally considering the same geographic areas associated with both of these issues/findings?

18,038 Posts
The Michigan Republican politicians in Lansing continue to support the captive cervid money interest over the welfare of human health, the Michigan deer herd, and the state tourism industry. They have threatened direct retaliation against any legislator that introduces anti cervid farm legislation.

Infectious prions found in deer meat
Research finds the matter, which causes chronic wasting disease, in muscle. It had been thought to be only in nervous-system tissue.
A person who eats venison could swallow the proteins shown to cause a deadly brain disease in deer, elk and moose, researchers reported today.
Their article in the journal Science represents the first time scientists have found the proteins that cause the affliction, chronic wasting disease, in the meat and muscle of deer.

New fears about deer http://www.jsonline.com/news/state/jan06/387848.asp

558 Posts
"Transgenic mice; the gene for the normal mouse version of the prion protein was replaced w/the normal protein for deer, so the mice made the normal, healthy deer protein.Infected tissue from infected deer was injected into the brains of these "transgenic" mice producing CWD lesions.
What this experiment proved is that muscle tissue from infected deer can infect deer, not humans
httpi//tinyurl.com/dzvo5; "Researchers at Case University showed that CWDwas not easily transmissible to humans; humanized mice genetically engineered to express the human form of normal prion protein in their brains were injected w/brain tissue samples from elk infected w/CWD. THE MICE DID NOT DEVELOP THE DISEASE However, mice that had been genetically engineered to express normal elk prion protein did get sick
Even if we should happen to consume venison that contains the abnormal prion protein, we will most likely,as the old proctologist told me when I was diagnosed w/prostate cancer, "you will probaly die from something else"

2,240 Posts
"you will probaly die from something else" Talk about spin.............

OK so MAYBE we won't get sick( maybe we will). Some of those deer are sick, so why should we risk the health of a wild deer herd for a few who choose to ignore the obvious?

164 Posts
I have quit hunting Wyoming and Colorado since my hunting areas went positive for cwd. I just could not bring venison or elk home and feed it to my daughters. They would be eating it without a choice or the knowledge to make a choice about its consumption. As a parent of grade school age children, we are responsible for their care.

As deer hunters, we must not do anything to encourage close contact of deer to each other and the spread of disease. We have discussed this topic before, so no more needs to be said. Escaped game farm cervidae are also a risk.

558 Posts
"Spin" is what makes the world go around. Most of our daily lives are influenced in some way by "Spin". Most of what we read (in newspapers, magazines, etc is "Spin". Most of what we hear on radio and also see on television is "Spin'. There is "good' Spin and there is "bad" Spin. "Spin" influences the outcome of national elections, not to mention getting us into international conflict. "Spin" is a necessary evil that lurks in the hearts of the Public Relations professional people (Spin Doctors). Without "Spin" how would we influence public perception, and energise our legislators to provide funding for programs and research before they return to their more important re-election campaigns
"Spin", like game farms, is here to stay. Without "Spin" and Spin Doctors it would be more difficult to obtain funding to combat diseases like BOVINE TB and CWD.
ps; will I die "with" or "from" prostate cancer when I die "from something else" ?

18,038 Posts
WI - New fears about deer

"This shows muscle contains infected material," said senior author Glenn Telling, an associate professor of microbiology at the University of Kentucky. "Anybody who may be handling or eating infected deer may be inadvertently exposed."
The finding does not change the state's recommendation for hunters, said James Kazmierczak, an epidemiologist with the Wisconsin Department of Health. No part of an infected animal should be consumed, and all animals killed in the chronic wasting disease endemic zone should be tested for the disease, he said.


1,446 Posts
Discussion Starter · #12 ·
>>>Even if we should happen to consume venison that contains the abnormal prion protein, we will most likely,as the old proctologist told me when I was diagnosed w/prostate cancer, "you will probaly die from something else"<<<

>>>"Spin" is what makes the world go around.<<<

let's spin this a bit. something to ponder for sure. but the incubation is what is fooling most. out of site, out of mind. but the TSE agent has NOW caught up to decades of cover-up. this is simple fact. it's all been very well documented in TEXAS with the infamous TEXAS mad cows that either do not get tested at all, or sit that sit up on the shelf for 4 to 8 months waiting for market to get right with GWs BSE MRR policy, the legal trading of all strains of TSE, the USA, being the Country with the most documented strains and species infected with TSE. something to be proud of for sure$

indeed, but apprx. 4.5 million anually die from Alzhiemer's too.
why is that important? about the only thing keep Alzhiemer's from being linked to a TSE is the myth that it will not transmit. however ;

CJD1/9 0185

Ref: 1M51A


Dr McGovern From: Dr A Wight

Date: 5 January 1993

Copies: Dr Metters

Dr Skinner

Dr Pickles

Dr Morris

Mr Murray


1. CMO will wish to be aware that a meeting was held at DH yesterday,
4 January, to discuss the above findings. It was chaired by Professor
Murray (Chairman of the MRC Co-ordinating Committee on Research in
the Spongiform Encephalopathies in Man), and attended by relevant
experts in the fields of Neurology, Neuropathology, molecular biology,
amyloid biochemistry, and the spongiform encephalopathies, and by
representatives of the MRC and AFRC.

2. Briefly, the meeting agreed that:

i) Dr Ridley et als findings of experimental induction of p amyloid
in primates were valid, interesting and a significant advance in the
understanding of neurodegeneradve disorders;

ii) there were no immediate implications for the public health, and no
further safeguards were thought to be necessary at present; and

iii) additional research was desirable, both epidemiological and at the
molecular level. Possible avenues are being followed up by DH
and the MRC, but the details will require further discussion.



BSE101/1 0136


5 NOV 1992

CMO From: Dr J S Metters DCMO 4 November 1992


1. Thank you for showing me Diana Dunstan's letter. I am glad that MRC have
recognised the public sensitivity of these findings and intend to report them in their
proper context. This hopefully will avoid misunderstanding and possible distortion by
the media to portray the results as having more greater significance than the findings
so far justify.

2. Using a highly unusual route of transmission (intra-cerebral injection) the
researchers have demonstrated the transmission of a pathological process from two
cases one of severe Alzheimer's disease the other of Gerstmann-Straussler disease to
marmosets. However they have not demonstrated the transmission of either clinical
condition as the "animals were behaving normally when killed'. As the report
emphasises the unanswered question is whether the disease condition would have
revealed itself if the marmosets had lived longer. They are planning funher research
to sec if the conditions, as opposed to the partial pathological process, is transmissible.

What are the implications for public health?

3. . The route of transmission is very specific and in the natural state of things
highly unusual. However it could be argued that the results reveal a potential risk,
in that brain tissue from these two patients has been shown to transmit a pathological
process. Should therefore brain tissue from such cases be regarded as potentially
infective? Pathologists, morticians, neuro surgeons and those assisting at neuro
surgical procedures and others coming into contact with "raw" human brain tissue
could in theory be at risk. However, on a priori grounds given the highly specific
route of transmission in these experiments that risk must be negligible if the usual
precautions for handling brain tissue are observed.


BSE101/1 0137

4. The other dimension to consider is the public reaction. To some extent the GSS
case demonstrates little more than the transmission of BSE to a pig by intra-cerebral
injection. If other prion diseases can be transmitted in this way it is little surprise that
some pathological findings observed m GSS were also transmissible to a marmoset.
But the transmission of features of Alzheimer's pathology is a different matter, given
the much greater frequency of this disease and raises the unanswered question whether
some cases are the result of a transmissible prion. The only tenable public line will
be that "more research is required" before that hypothesis could be evaluated. The
possibility on a transmissible prion remains open. In the meantime MRC needs
carefully to consider the range and sequence of studies needed to follow through from
the preliminary observations in these two cases. Not a particularly comfortable
message, but until we know more about the causation of Alzheimer's disease the total
reassurance is not practical.

Room 509
Richmond House
Pager No: 081-884 3344
Callsign: DOH 832




By Terry Singletary, Sr

Note - This extensive, powerful assemblage of science was first posted on 1-24-3. The
following data is even more important today. -ed


Regarding Alzheimer's disease

(note the substantial increase on a yearly basis)



The pathogenesis of these diseases was compared to Alzheimer's disease at a molecular level...



And NONE of this is relevant to BSE?

There is also the matter whether the spectrum of ''prion disease'' is wider than that recognized at present.


Human BSE


These are not relevant to any possible human hazard from BSE nor to the much more common dementia, Alzheimers.



NOW, let us look and see what the CDC et al really have to say about CWD to humans ;


Chronic Wasting Disease and Potential Transmission to Humans
Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,&#8224; Michael W. Miller,&#8225; Pierluigi Gambetti,&#167; and Lawrence B. Schonberger*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA; &#8224;University of Wyoming, Laramie, Wyoming, USA; &#8225;Colorado Division of Wildlife, Fort Collins, Colorado, USA; and &#167;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.


Risk for Transmission to Humans
Epidemiologic Studies
The increasing detection of CWD in a wider geographic area and the presumed foodborne transmission of BSE to humans, resulting in cases of vCJD, have raised concerns about the possible zoonotic transmission of CWD (32). In the late 1990s, such concerns were heightened by the occurrence of CJD among three patients 30 years of age who were deer hunters or ate deer and elk meat harvested by family members (Table 2). However, epidemiologic and laboratory investigations of these case-patients indicated no strong evidence for a causal link between CWD and their CJD illness (33). None of the patients were reported to have hunted deer or eaten deer meat harvested in the CWD-endemic areas of Colorado and Wyoming. Such a history in unusually young CJD patients, if present, would have supported a causal link with CWD. Moreover, the testing of brain tissues from >1,000 deer and elk harvested from areas where the patients hunted or their venison originated did not show any evidence of CWD (33). In addition, the lack of homogeneity in the clinicopathologic manifestation and codon 129 of the prion protein gene among the three patients suggested that their illnesses could not be explained by exposure to the same prion strain. In vCJD, homogeneity of the genotype at codon 129 and the clinical and pathologic phenotype were attributed to the patients' exposure to the same prion strain, the agent of BSE.

In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting &#8776;22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Str&#228;ussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease.

In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5&#8211;6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient's disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36).

The second patient died from an illness lasting <16 months. The patient's illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient's death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient's parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1&#8211;2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin.

Recently, rare neurologic disorders resulting in the deaths of three men who participated in "wild game feasts" in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick's disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming.

In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35).

To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient's CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient's illness was consistent with the MM1 CJD phenotype.

Despite the decades-long endemicity of CWD in Colorado and Wyoming, the incidence of CJD and the age distribution of CJD case-patients in these two states are similar to those seen in other parts of the United States. From 1979 to 2000, 67 CJD cases from Colorado and 7 from Wyoming were reported to the national multiple cause-of-death database. The average annual age-adjusted CJD death rate was 1.2 per million persons in Colorado and 0.8 in Wyoming. The proportion of CJD patients who died before age 55 in Colorado (13.4%) was similar to that of the national (10.2%). The only CJD case-patient <30 years of age in Colorado had iatrogenic CJD linked to receipt of human growth hormone injections. CJD was not reported in persons <55 years of age in Wyoming during the 22-year surveillance period.



1,446 Posts
Discussion Starter · #13 ·
The lack of evidence of a link between CWD transmission and unusual cases of CJD, despite several epidemiologic investigations, and the absence of an increase in CJD incidence in Colorado and Wyoming suggest that the risk, if any, of transmission of CWD to humans is low. Although the in vitro studies indicating inefficient conversion of human prion protein by CWD-associated prions raise the possibility of low-level transmission of CWD to humans, no human cases of prion disease with strong evidence of a link with CWD have been identified. However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease. The level and frequency of human exposure to the CWD agent may increase with the spread of CWD in the United States. Because the number of studies seeking evidence for CWD transmission to humans is limited, more epidemiologic and laboratory studies should be conducted to monitor the possibility of such transmissions. Studies involving transgenic mice expressing human and cervid prion protein are in progress to further assess the potential for the CWD agent to cause human disease. Epidemiologic studies have also been initiated to identify human cases of prion disease among persons with an increased risk for exposure to potentially CWD-infected deer or elk meat (47). If such cases are identified, laboratory data showing similarities of the etiologic agent to that of the CWD agent would strengthen the conclusion for a causal link. Surveillance for human prion diseases, particularly in areas where CWD has been detected, remains important to effectively monitor the possible transmission of CWD to humans. Because of the long incubation period associated with prion diseases, convincing negative results from epidemiologic and experimental laboratory studies would likely require years of follow-up. In the meantime, to minimize the risk for exposure to the CWD agent, hunters should consult with their state wildlife agencies to identify areas where CWD occurs and continue to follow advice provided by public health and wildlife agencies. Hunters should avoid eating meat from deer and elk that look sick or test positive for CWD. They should wear gloves when field-dressing carcasses, bone-out the meat from the animal, and minimize handling of brain and spinal cord tissues. As a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified.



AS with the BSE TO HUMANS AND BSE to GOAT. IT was always it never happend under natural conditions, just in the lab, so not to worry. NOW WE HAVE TO WORRY;



CWD to CJD in humans (why not?), as easy as BSE/Scrapie;

The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization

Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
and B. Caughey1,7

1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: [email protected] Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.


Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.


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.



EXACTLY what did the CDC et al say about CWD to humans in the USA ;

From: TSS (216-119-163-189.ipset45.wt.net)
Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"
Cc: "Race, Richard (NIH)" ; ; "Belay,
Sent: Monday, September 30, 2002 9:22 AM

> Dear Sir/Madam,
> In the Archives of Neurology you quoted (the abstract of which was


> to your email), we did not say CWD in humans will present like variant


> 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: [email protected]; [email protected]; [email protected]





Issued: Monday, 28 August 2000 NEW EVIDENCE OF SUB-CLINICAL PRION

A team of researchers led by Professor John Collinge at the Medical
Research Council Prion Unit1 report today in the Proceedings of the
National Academy of Sciences, on new evidence for the existence of a
'sub-clinical' form of BSE in mice which was unknown until now.

The scientists took a closer look at what is known as the 'species
barrier' - the main protective factor which limits the ability of
prions2 to jump from one species to infect another. They found the mice
had a 'sub-clinical' form of disease where they carried high levels of
infectivity but did not develop the clinical disease during their normal
lifespan. The idea that individuals can carry a disease and show no
clinical symptoms is not new. It is commonly seen in conventional
infectious diseases.

Researchers tried to infect laboratory mice with hamster prions3 called
Sc237 and found that the mice showed no apparent signs of disease.
However, on closer inspection they found that the mice had high levels
of mouse prions in their brains. This was surprising because it has
always been assumed that hamster prions could not cause the disease in
mice, even when injected directly into the brain.

In addition the researchers showed that this new sub-clinical infection
could be easily passed on when injected into healthy mice and hamsters.

The height of the species barrier varies widely between different
combinations of animals and also varies with the type or strain of
prions. While some barriers are quite small (for instance BSE easily
infects mice), other combinations of strain and species show a seemingly
impenetrable barrier. Traditionally, the particular barrier studied here
was assumed to be robust.

Professor John Collinge said: "These results have a number of important
implications. They suggest that we should re-think how we measure
species barriers in the laboratory, and that we should not assume that
just because one species appears resistant to a strain of prions they
have been exposed to, that they do not silently carry the infection.
This research raises the possibility, which has been mentioned before,
that apparently healthy cattle could harbour, but never show signs of, BSE.

"This is a timely and unexpected result, increasing what we know about
prion disease. These new findings have important implications for those
researching prion disease, those responsible for preventing infected
material getting into the food chain and for those considering how best
to safeguard health and reduce the risk that theoretically, prion
disease could be contracted through medical and surgical procedures."



NOW, look at the steady increase in the sporadic CJDs in the USA, and most importantly, or most disturbing, is the phenotypes that are now being documented that is a 'unknown phenotype' ;


Animal Prion Diseases Relevant to Humans (unknown types?)
Thu Oct 27, 2005 12:05

About Human Prion Diseases /
Animal Prion Diseases Relevant to Humans

Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.

Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)

Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)



1,446 Posts
Discussion Starter · #14 ·
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here;

p.s. please note the 47 PENDING CASES to Sept. 2005

p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ???

p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???

p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN???



AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.





Date: January 12, 2006 at 11:29 am PST

Infected and Source Flocks

As of November 30, 2005 there were 95 scrapie infected and source flocks (Figure 3). There were 2 new infected and source flocks reported in November (Figure 4) with a total of 12 flocks reported for FY 2006 (Figure 5). The total infected and source flocks that have been released in FY 2006 are 16 (Figure 6), with 9 flocks released in November. The ratio of infected and source flocks released to newly infected and source flocks for FY 2006 = 1.33 : 1. In addition, as of November 30, 2005, 67 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 7 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in November 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005. New infected flocks, source flocks, and flocks released for FY 2006 are depicted in Figure 10. New infected and source statuses from 1997 to 2006 are depicted in Chart 3.

Regulatory Scrapie Slaughter Surveillance (RSSS) +

RSSS started April 1, 2003. It is targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. Samples have been collected from 67,840 sheep since April 1, 2003, of which results have been reported for 64,034. Samples have been submitted from 81 plants. There have been 215 NVSL confirmed positive sheep since the beginning of RSSS. In FY 2006 samples have been collected from 5,339 sheep and there have been 7 NVSL confirmed positive cases through November 2005. Face colors of FY 2006 confirmed positives are 6 black and 1 mottled. During November 2005, 2,429* animals were sampled and test results were reported on 3088 samples. Five confirmed positives were reported by NVSL in November 2005. Cumulative regional sample collection numbers are shown in Figure 11 and are based upon the State in which the animal was tagged. The number of RSSS animals collected with traceable identification for FY 2005 by month, by region where collected is shown in Figure 12. A retrospective 6 month rolling average of the % positive tested black-faced sheep sampled at slaughter is shown in Figure 13.


full text;


Office Note


A The Present Position with respect to Scrapie
A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all

The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.

It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.

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

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




Published online before print October 20, 2005

Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
Medical Sciences

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

( sheep prion | transgenic mice )

Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
*Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

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


Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper.

A.L.D. and V.B. contributed equally to this work.

To whom correspondence should be addressed.

Hubert Laude, E-mail: [email protected]



Subject: Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)
Date: October 19, 2005 at 8:33 am PST

0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

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.
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

Received 3 May 2005/ Accepted 10 August 2005

Chronic 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


* 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:
[email protected] .



Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21
0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.


i suppose we will just die old and demented, and pray we get a very fast strain, such as the sCJD's. ...TSS:bash: :bash: :bash:

267 Posts
Scientists is people too. Ya must remember that there is wackos out there in all occupations trying to find ways ta shut down hunting, trappin, and fishin, includin scientists. Meny of them is hell bent on tryin ta get hunters outta the woods so that things will be natural again, according to their view of natural.

This is were the new antihuntin battle is headed...the "scientific arena". They is trying to "create" scientific data ta shut down huntin.:help:

495 Posts
I can't tell if the previous post is real or a spoof so I will treat it as real but ignorant as death and disease is not something that I joke about. If you are convinced that CWD or TSE is some big hoax or scientific conspiracy then I'm sure that we can come up with all the meat you want from one of these scientific prank CWD deer for your dinner table and freezer. I think it is delusional and in extremely bad taste to post and to think that anti hunting groups would purposely delude the world regarding disease and it's ramifications. Again, I'm more than willing to pass along any or all CWD positive deer to you for your dinner table if your convinced that CWD is some contrived malady of no consequence.

558 Posts
The 2005 Wildlife Disease Committee Report, also the 2005 TB Committee Report, are now available on the US Animal Health Association page. Some very interesting information, such as the fact that the CWD PRION HAS BEEN FOUND IN THE HEART MUSCLE OF W/TAIL DEER AND ELK
USAHA News Alerts provide daily info on newly emerging diseases
Good "Spin"

267 Posts
Ogre said:
I can't tell if the previous post is real or a spoof so I will treat it as real but ignorant as death and disease is not something that I joke about. If you are convinced that CWD or TSE is some big hoax or scientific conspiracy then I'm sure that we can come up with all the meat you want from one of these scientific prank CWD deer for your dinner table and freezer. I think it is delusional and in extremely bad taste to post and to think that anti hunting groups would purposely delude the world regarding disease and it's ramifications. Again, I'm more than willing to pass along any or all CWD positive deer to you for your dinner table if your convinced that CWD is some contrived malady of no consequence.
i never said it aint a real disease or concern. what i did say is that there is animal rights wackos and anti huntin scientists that will use whatevr they can to try to stop huntin.

what if scientests did a study and said that people huntin deer was leadin to diseases like CWD. some scientists think man huntin aint natural. some scientests want people ta believe that wolves, bear, and cougars should only be the ones ta hunt deer cause they go for the weakest deer.

i is being serious on this. i has read studys by scientists who are tryin to make the case that man shouldnt hunt. they back this with "science".

1,446 Posts
Discussion Starter · #19 ·
>>>> let's spin this a bit. something to ponder for sure. but the incubation is what is fooling most. out of site, out of mind. but the TSE agent has NOW caught up to decades of cover-up. this is simple fact. it's all been very well documented in TEXAS with the infamous TEXAS mad cows that either do not get tested at all, or sit that sit up on the shelf for 4 to 8 months waiting for market to get right with GWs BSE MRR policy, the legal trading of all strains of TSE, the USA, being the Country with the most documented strains and species infected with TSE. something to be proud of for sure$<<<<

some might have thought i was kidding, if so, maybe you read this ;

Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II and Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL Washington, D.C. 20250 January 25, 2006 REPLY TO ATTN OF: 50601-10-KC TO: W. Ron DeHaven Administrator Animal and Plant Health Inspection Service Barbara Masters Administrator Food Safety and Inspection Service ATTN: William J. Hudnall Deputy Administrator Marketing Regulatory Program Business Services William C. Smith Assistant Administrator Office of Program Evaluation, Enforcement, and Review FROM: Robert W. Young /s/ Assistant Inspector General for Audit SUBJECT: Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III This report presents the results of our audit of the enhanced BSE surveillance program and controls over specified risk materials and advanced meat recovery products. Your written response to the official draft report, dated January 20, 2006, is included as exhibit G with excerpts of the response and the Office of Inspector General’s (OIG) position incorporated into the Findings and Recommendations section of the report, where applicable. We accept the management decisions for all recommendations. Please follow your agency’s internal procedures in forwarding documentation for final action to the Office of the Chief Financial Officer (OCFO). We are providing a separate memorandum to the agencies and OCFO that provides specific information on the actions to be completed to achieve final action. We appreciate your timely response and the cooperation and assistance provided to our staff during the audit USDA/OIG-A/50601-10-KC/ Page i

Executive Summary

Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Results in Brief This report evaluates elements of the interlocking safeguards in place to protect United States (U.S.) beef from Bovine Spongiform Encephalopathy, widely known as BSE or "mad cow disease." Since 1990, the U.S. Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), has led a multi-agency effort to monitor and prevent BSE from entering the food supply. After discovering a BSE-positive cow in December 2003, APHIS expanded its BSE surveillance program. To further protect the food supply, USDA banned materials identified as being at risk of carrying BSE (specified risk materials (SRM)), such as central nervous system tissue. As part of this effort, USDA’s Food Safety and Inspection Service (FSIS) required beef slaughter and processing facilities to incorporate controls for handling such materials into their operational plans. Onsite FSIS inspectors also inspect cattle for clinical signs in order to prevent diseased animals from being slaughtered for human consumption. To evaluate the effectiveness of the safeguards, we assessed APHIS’ implementation of the expanded surveillance program, as well as FSIS’ controls to prevent banned SRMs from entering the food supply.

In June 2004, APHIS implemented its expanded surveillance program; participation by industry in this surveillance program is voluntary. As of May 2005, over 350,000 animals were sampled and tested for BSE. To date, two animals tested positive for BSE; one tested positive after implementation of the expanded surveillance program.

USDA made significant efforts to implement the expanded BSE surveillance program. Much needed to be done in a short period of time to establish the necessary processes, controls, infrastructure, and networks to assist in this effort. In addition, extensive outreach and coordination was undertaken with other Federal, State, and local entities, private industry, and laboratory and veterinary networks. This report provides an assessment as to the progress USDA made in expanding its surveillance effort and the effectiveness of its controls and processes. This report also discusses the limitations of its program and data in assessing the prevalence of BSE in the U.S. herd.


40 ELISA test procedures require two additional (duplicate) tests if the initial test is reactive, before final interpretation. If either of the duplicate tests is reactive, the test is deemed inconclusive.

41 Protocol for BSE Contract Laboratories to Receive and Test Bovine Brain Samples and Report Results for BSE Surveillance Standard Operating Procedure (SOP), dated October 26, 2004.

42 The NVSL conducted an ELISA test on the original material tested at the contract laboratory and on two new cuts from the sample tissue.

43 A visual examination of brain tissue by a microscope.

44 A localized pathological change in a bodily organ or tissue.



PAGE 43;

Section 2. Testing Protocols and Quality Assurance Controls





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267 Posts

you is scarin me with all of that mumbo jumbo bout mad cows in texas. NOT! funny how when the beef market has hit the highest all time prices ever that mad cows start poppin up evry where.

just the other day McDonalds officials were jumpin the goverments butt bout the beef supply not bein safe. now just stop and thank a minute, if you is selling hamburgers why would ya make a big media release saying beef aint safe? simple, they is losin money due ta sky high beef prices. they know that people is still gonna eat hamburgers so they is tryin ta drive down the market some.

japan opens up its markets again and sure enough the first shipment ta japan contains spinal cord material.:tdo12: the prices had jumped due ta japan importin beef again and it amazinly fell right back ta where it was.

i has also red a study where mice eating infected beef never came down with the disease. in this same study they also injected bse directly into the brains of mice. only 10% contracted it this way.

i guess we shou9ld be tremblin with fear....we is all gonna die.(someday):lol:
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