# Atypical Prion Diseases in Humans and Animals 2011



## terry (Sep 13, 2002)

Monday, May 23, 2011 


Atypical Prion Diseases in Humans and Animals 2011 


Top Curr Chem (2011)


DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011


Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar





Abstract


Although prion diseases, such as CreutzfeldtJakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the scrapie form (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.


M.A. Tranulis (*)

Norwegian School of Veterinary Science, Oslo, Norway

e-mail: [email protected]

S.L. Benestad

Norwegian Veterinary Institute, Oslo, Norway

T. Baron

Agence Nationale de Se´curite´ Sanitaire, ANSES, Lyon, France

H. Kretzschmar

LudwigMaximilians University of Munich, Munich, Germany


Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type



http://resources.metapress.com/pdf-preview.axd?code=f433r34h34ugg617&size=largest




snip...SEE MORE HERE ;




http://bse-atypical.blogspot.com/2011/05/atypical-prion-diseases-in-humans-and.html






TSS


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## 7mmsendero (Dec 2, 2010)

One aspect of prion diseases and CWD that I've wondered about is the potential for a "canary in the mine" that could help us accelerate our understanding of the risks.

Here is the thing, predators are exposed to CWD at much greater rates than humans. Bears, coyotes, wolves, and cougars must be killing and consuming large numbers of CWD infected animals. Additionally, these predators are consuming high risk animal parts anytime a kill is made (organs, nevous system tissues, bone marrow). So, are we currently looking for a bear, wolf, coyote, or cougar that is infected with a brain disease? 

I remember talk of this a decade ago, however finding any type of an update is difficult.

Maybe these species do not get prion related brain diseases, so it could be a moot point. However, if CWD could make a jump, it is reasonable to assume it will happen among these predators much sooner.


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## terry (Sep 13, 2002)

7mmsendero said:


> One aspect of prion diseases and CWD that I've wondered about is the potential for a "canary in the mine" that could help us accelerate our understanding of the risks.
> 
> snip.





the canaries in the coal mine of TSE prion disease began dying 3 decades ago or so. from species to species the canaries in the coal have been dying off for some time, while government and industry officials turned a blind eye, and then came up with the grandest of schemes. the UKBSEnvCJD only theory. thus, an epidemic was born. 



Monday, February 14, 2011 


THE ROLE OF PREDATION IN DISEASE CONTROL: A COMPARISON OF SELECTIVE AND NONSELECTIVE REMOVAL ON PRION DISEASE DYNAMICS IN DEER


snip...


Greetings again JWD et al,

AS a layperson, but one that has followed the TSE saga since 1997 daily, after the loss of my Mother to the Heidenhain Variant of Creutzfeldt Jakob Disease, I would kindly like to make the following comments please.

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

"We suggest that as CWD distribution and wolf range overlap in the future, wolf predation may suppress disease emergence or limit prevalence."

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

NEGATIVE, NEGATIVE, NEGATIVE, you cannot eradicate or control cwd by spreading to other species, and or letting them consume infected carcasses to spread infection miles away by shedding via feces, urine, etc. to the environment. Plus, if you look at old transmission studies i.e. the 'hound study', you will see that there were strange pathology in transmission studies.

i kindly disagree using wolf to try and eradicate CWD for the following reasons.


snip...please see positive results on hound survey ;


http://chronic-wasting-disease.blogspot.com/2011/02/role-of-predation-in-disease-control.html




Tuesday, November 03, 2009 

Mountain lions prey selectively on prion-infected mule deer


Seems prudent now, to do studies of prion disease of mountain lions in CWD zones. But that probably makes to much sense.

ALSO, what about mountain lion feces after consuming a CWD infected mule deer. a good healthy dump of prions I suppose, for the environment. ...TSS


SNIP...


Subject: FSE: FIRST CONFIRMED CASE REPORTED IN PORTUGAL AND POTENTIAL MAD CAT ESCAPES LAB IN USA Date: August 9, 2007 at 2:27 pm PST


http://chronic-wasting-disease.blogspot.com/2009/11/mountain-lions-prey-selectively-on.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



Chronic Wasting Disease Susceptibility of Four North American Rodents


We intracerebrally challenged four species of native North American rodents that inhabit locations undergoing cervid chronic wasting disease (CWD) epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles (Myodes gapperi). The inocula were prepared from the brains of hunter-harvested white-tailed deer from Wisconsin hunting that tested positive for CWD. Meadow voles proved to be most susceptible, with a median incubation period of 272 days. Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the brains of all challenged meadow voles. Subsequent passages in meadow voles lead to a significant reduction in incubation period. The disease progression in red-backed voles, which are very closely related to the European bank vole (M. glareolus) which have been demonstrated to be sensitive to a number of TSEs, was slower than in meadow voles with a median incubation period of 351 days. We sequenced the meadow vole and red-backed vole Prnp genes and found three amino acid (AA) differences outside of the signal and GPI anchor sequences. Of these differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is particularly intriguing due its postulated involvement in "rigid loop" structure and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5 years post-inoculation, but appear to be exhibiting a high degree of disease penetrance. White-footed mice have an even longer incubation period but are also showing high penetrance. Second passage experiments show significant shortening of incubation periods. Meadow voles in particular appear to be interesting lab models for CWD. These rodents scavenge carrion, and are an important food source for many predator species. Furthermore, these rodents enter human and domestic livestock food chains by accidental inclusion in grain and forage. Further investigation of these species as potential hosts, bridge species, and reservoirs of CWD is required.

Potential Venison Exposure Among FoodNet Population Survey Respondents, 2006-2007

Ryan A. Maddox1*, Joseph Y. Abrams1, Robert C. Holman1, Lawrence B. Schonberger1, Ermias D. Belay1 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vector-Borne, and Enteric Diseases, Centers for Disease Control and Prevention, Atlanta, GA *Corresponding author e-mail: [email protected]

The foodborne transmission of bovine spongiform encephalopathy to humans, resulting in variant Creutzfeldt-Jakob disease, indicates that humans can be susceptible to animal prion diseases. However, it is not known whether foodborne exposure to the agent causing chronic wasting disease (CWD) in cervids can cause human disease. The United States Foodborne Diseases Active Surveillance Network (FoodNet) conducts surveillance for foodborne diseases through an extensive survey administered to respondents in selected states. To describe the frequency of deer and elk hunting and venison consumption, five questions were included in the 2006-2007 FoodNet survey. This survey included 17,372 respondents in ten states: California, Colorado, Connecticut, Georgia, Maryland, Minnesota, New Mexico, New York, Oregon, and Tennessee. Of these respondents, 3,220 (18.5%) reported ever hunting deer or elk, with 217 (1.3%) reporting hunting in a CWD-endemic area (northeastern Colorado, southeastern Wyoming, and southwestern Nebraska). Of the 217 CWD-endemic area hunters, 74 (34.1%) were residents of Colorado. Respondents reporting hunting were significantly more likely to be male than female (prevalence ratio: 3.3, 95% confidence interval: 3.1-3.6) and, in general, older respondents were significantly more likely to report hunting than younger respondents. Venison consumption was reported by more than half (67.4%) of the study population, and most venison consumers (94.1%) reported that at least half of their venison came from the wild. However, more than half (59.1%) of the consumers reported eating venison only one to five times in their life or only once or twice a year. These findings indicate that a high percentage of the United States population engages in hunting and/or venison consumption. If CWD continues to spread to more areas across the country, a substantial number of people could potentially be exposed to the infectious agent.

http://www.cwd-info.org/pdf/3rd_CWD_Symposium_utah.pdf



http://chronic-wasting-disease.blogspot.com/2010/12/cwd-update-99-december-13-2010.html



http://transmissiblespongiformencep...11/05/travel-history-hunting-and-venison.html



http://transmissiblespongiformencep...05/cdc-assesses-potential-human-exposure.html





AS THE CROW FLIES, SO DO TSE'S

Sunday, November 01, 2009

American crows (Corvus brachyrhynchos) and potential spreading of CWD through feces of digested infectious carcases

http://chronic-wasting-disease.blogspot.com/2009/11/american-crows-corvus-brachyrhynchos.html



Wednesday, February 16, 2011

IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE


snip...


reference...

RB3.20

TRANSMISSION TO CHIMPANZEES

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

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

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

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

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

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

R. Bradley

23 September 1990

CVO (+Mr Wells' comments)

Dr T W A Little

Dr B J Shreeve

90/9.23/1.1.

http://collections.europarchive.org...einquiry.gov.uk/files/yb/1990/09/23001001.pdf


snip...


http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html





Feline Spongiform Encephalopathy (FSE)

FSE was first identified in the UK in 1990. Most cases have been reported in the UK, where the epidemic has been consistent with that of the BSE epidemic. Some other countries (e.g. Norway, Liechtenstein and France) have also reported cases.

Most cases have been reported in domestic cats but there have also been cases in captive exotic cats (e.g. Cheetah, Lion, Asian leopard cat, Ocelot, Puma and Tiger). The disease is characterised by progressive nervous signs, including ataxia, hyper-reactivity and behavioural changes and is fatal.

The chemical and biological properties of the infectious agent are identical to those of the BSE and vCJD agents. These findings support the hypothesis that the FSE epidemic resulted from the consumption of food contaminated with the BSE agent.

The FSE epidemic has declined as a result of tight controls on the disposal of specified risk material and other animal by-products. &#8226; References:

Leggett, M.M. et al.(1990) A spongiform encephalopathy in a cat. Veterinary Record. 127. 586-588

Synge, B.A. et al. (1991) Spongiform encephalopathy in a Scottish cat. Veterinary Record. 129. 320

Wyatt, J. M. et al. (1991) Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats. Veterinary Record. 129. 233.

Gruffydd-Jones, T. J.et al.. (1991) Feline spongiform encephalopathy. J. Small Animal Practice. 33. 471-476.

Pearson, G. R. et al. (1992) Feline spongiform encephalopathy: fibril and PrP studies. Veterinary Record. 131. 307-310.

Willoughby, K. et al. (1992) Spongiform encephalopathy in a captive puma (Felis concolor). Veterinary Record. 131. 431-434.

Fraser, H. et al. (1994) Transmission of feline spongiform encephalopathy to mice. Veterinary Record 134. 449.

Bratberg, B. et al. (1995) Feline spongiform encephalopathy in a cat in Norway. Veterinary Record 136. 444

Baron, T. et al. (1997) Spongiform encephalopathy in an imported cheetah in France. Veterinary Record 141. 270-271

Zanusso, G et al. (1998) Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy. Lancet, V352, N9134, OCT 3, Pp 1116-1117.

Ryder, S.J. et al. (2001) Inconsistent detection of PrP in extraneural tissues of cats with feline spongiform encephalopathy. Veterinary Record 146. 437-441

Kelly, D.F. et al. (2005) Neuropathological findings in cats with clinically suspect but histologically unconfirmed feline spongiform encephalopathy. Veterinary Record 156. 472-477.



TSEs in Exotic Ruminants


CONTINUED IN PART 2 TO FOLLOW ;


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## terry (Sep 13, 2002)

[/QUOTE]TSEs in Exotic Ruminants


CONTINUED IN PART 2 TO FOLLOW ;[/QUOTE]




PART 2 ;


TSEs in Exotic Ruminants

TSEs have been detected in exotic ruminants in UK zoos since 1986. These include antelopes (Eland, Gemsbok, Arabian and Scimitar oryx, Nyala and Kudu), Ankole cattle and Bison. With hindsight the 1986 case in a Nyala was diagnosed before the first case of BSE was identified. The TSE cases in exotic ruminants had a younger onset age and a shorter clinical duration compared to that in cattle with BSE. All the cases appear to be linked to the BSE epidemic via the consumption of feed contaminated with the BSE agent. The epidemic has declined as a result of tight controls on feeding mammalian meat and bone meal to susceptible animals, particularly from August 1996.  References:

Jeffrey, M. and Wells, G.A.H, (1988) Spongiform encephalopathy in a nyala (Tragelaphus angasi). Vet.Path. 25. 398-399

Kirkwood, J.K. et al (1990) Spongiform encephalopathy in an Arabian oryx (Oryx leucoryx) and a Greater kudu (Tragelaphus strepsiceros) Veterinary Record 127. 418-429.

Kirkwood, J.K. (1993) Spongiform encephalopathy in a herd of Greater kudu (Tragelaphus strepsiceros): epidemiological observations. Veterinary Record 133. 360-364

Kirkwood, J. K. and Cunningham, A.A. (1994) Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Veterinary Record. 135. 296-303.

Food and Agriculture Organisation (1998) Manual on Bovine Spongiform Encephalopathy.


http://archive.defra.gov.uk/foodfarm/farmanimal/diseases/atoz/bse/othertses/#exotic



Zoo Prion Disease: Review of Scientific Literature


http://www.mad-cow.org/zoo_cites_annotated.html



Prions in primates and other zoo animals 22 April 1999. Contact webmaster with additions or corrections. fax 541-484-0669 US Four important papers by Noelle Bons and coworkers concerning TSE in primates are summarized on a separate page. The table below summarizes results in the 1999 PNAS paper. Penetrance of the disease is very high and many animals did not yet display symptoms . This paper was the first (and only one) to look at non-symptomatic zoo animals for prion infection (shown below in red). In the TSE column of the table, '+' signs indicate confirmed, 'p' indicates suspicious/probable, '-' means CNS study negative for TSE.(shown as brown), 'pc' means positive diagnosis in preclinical animal.

PNAS 96:4046-4051 199 30 Mar 1999 full text see comment PNAS 96[9] 4738-4739, April 27, 1999 by Will and Ironside C R Acad Sci III 1997 Dec;320(12):971-9 N Bons et al. C R Acad Sci III 1996 Aug;319(8):733-6 Lancet Volume 348, Number 9019 6 July 1996

The 82 zoo animals with BSE:

Id TSE Genus Species Subsp Birth Origin Death Place of Death 654 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier 656 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier 481 + Eulemur fulvus mayottensis 1974 Madagascar 1992 Montpellier zoo 474 + Eulemur fulvus mayottensis 1974 Madagascar 1990 Montpellier zoo 584 - Eulemur fulvus mayottensis 1984 Montpellier 1991 Montpellier zoo 455 + Eulemur fulvus mayottensis 1983 Montpellier 1989 Montpellier zoo - + Eulemur fulvus mayottensis 1988 Montpellier 1992 Montpellier zoo - + Eulemur fulvus mayottensis 1995 Montpellier 1996 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo 456 + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo 586 + Eulemur mongoz - 1979 Madagascar 1998 Montpellier zoo - p Eulemur mongoz - 1989 Mulhouse 1991 Montpellier zoo - p Eulemur mongoz - 1989 Mulhouse 1990 Montpellier zoo - p Eulemur macaco - 1986 Montpellier 1996 Montpellier zoo - p Lemur catta - 1976 Montpellier 1994 Montpellier zoo - p Varecia variegata variegata 1985 Mulhouse 1990 Montpellier zoo - p Varecia variegata variegata 1993 xxx 1994 Montpellier zoo 455 + Macaca mulatta - 1986 Ravensden UK 1992 Montpellier zoo - p Macaca mulatta - 1986 Ravensden UK 1993 Montpellier zoo - p Macaca mulatta - 1988 Ravensden UK 1991 Montpellier zoo - p Saimiri sciureus - 1987 Frejus France 1990 Frejus zoo 700 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 701 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 702 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 703 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 704 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 705 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo 706 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 707 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 708 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 709 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 710 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 711 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 712 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 713 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 714 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 715 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 716 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo 717 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo x p genus species - - Lille zoo 1996 Lille zoo y p genus species - - Lille zoo 1996 Lille zoo z p genus species - - Lille zoo 1996 Lille zoo

1 + Actinonyx jubatus cheetah 1986 Marwell zoo 1991 Pearle Coast AU Duke + Actinonyx jubatus cheetah 1984 Marwell zoo 1992 Colchester zoo? UK Saki + Actinonyx jubatus cheetah 1986 Marwell zoo 1993 unknown UK Mich + Actinonyx jubatus cheetah 1986 Whipsnade 1993 Whipsnade UK Fr1 + Actinonyx jubatus cheetah 1987 Whipsnade 1997 Safari de Peaugres FR Fr2 + Actinonyx jubatus cheetah 1991 Marwell zoo 1997 Safari de Peaugres Fr xx + Actinonyx jubatus cheetah 19xx xxx zoo 199x Fota zoo IR yy + Actinonyx jubatus cheetah 19xx yyy zoo 1996+ yyyy zoo UK zz + Actinonyx jubatus cheetah 19xx zzz zoo 1996+ yyyy zoo UK

aaa + Felis concolor puma 1986 Chester zoo 1991 Chester zoo UK yy + Felis concolor puma 1980 yyy zoo 1995 yyyy zoo UK zz + Felis concolor puma 1978 zzz zoo 1995 zzzz zoo UK

xxx + Felis pardalis ocelot 1987 xxx 1994 Chester zoo UK zzz + Felis pardalis ocelot 1980 zzz 1995 zzzz zoo UK

85 + Felis catus cat 1990+ various 1999+ various UK LI NO 19 + Canis familia. dog 1992+ various 1999+ various UK

Fota + Panthera tigris tiger 1981 xxx zoo 1995 xxxx zoo UK yy + Panthera tigris tiger 1983 yyy zoo 1998 yyyy zoo UK

Lump + Panthera leo lion 1986 Woburn SP 1998 Edinburgh zoo UK [since 1994]

1 + Taurotragus oryx eland 1987 Port Lympne 1989 Port Lympne zoo UK Moll + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK Nedd + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK Elec + Taurotragus oryx eland 1990 xx UK 1992 not Port Lympne Uk Daph p Taurotragus oryx eland 1988 xx UK 1990 not Port Lympne UK zzz + Taurotragus oryx eland 1991 zz UK 1994 zzz UK yyy + Taurotragus oryx eland 1993 yy UK 1995 yyy UK

Fran p Tragelaphus strepsi. kudu 1985 London zoo 1987 London zoo UK Lind + Tragelaphus strepsi. kudu 1987 London zoo 1989 London zoo UK Karl + Tragelaphus strepsi. kudu 1988 London zoo 1990 London zoo UK Kaz + Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK Bamb pc Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK Step - Tragelaphus strepsi. kudu 1984 London zoo 1991 London zoo UK 346 pc Tragelaphus strepsi. kudu 1990 London zoo 1992 London zoo UK 324 + Tragelaphus strepsi. kudu 1989 Marwell zoo 1992 London zoo UK

xxx + Tragelaphus angasi nyala 1983 Marwell zoo 1986 Marwell zoo UK

yy + Oryx gazella gemsbok 1983 Marwell zoo 1986 Marwell zoo UK zz + Oryx gazella gemsbok 1994+ zzz zoo 1996+ zzzz zoo UK

xx + Oryx dammah scim oryx 1990 xxxx zoo 1993 Chester zoo UK

yy + Oryx leucoryx arab oryx 1986 Zurich zoo  1991 London zoo UK

yy + Bos taurus ankole cow 1987 yyy zoo 1995 yyyy zoo UK zz + Bos taurus ankole cow 1986 zzz zoo 1991 zzzz zoo UK

xx + Bison bison Eu bison 1989 xxx zoo 1996 xxxx zoo UK



http://www.mad-cow.org/zoo_cites_annotated.html


Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html


Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html



Thursday, April 28, 2011

Chronic Wasting Disease Testing and Prevalence Wisconsin April 2011

http://chronic-wasting-disease.blogspot.com/2011/04/chronic-wasting-disease-testing-and.html


UPDATED DATA ON 2ND CWD STRAIN

Wednesday, September 08, 2010

CWD PRION CONGRESS SEPTEMBER 8-11 2010

http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html


Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

http://chronic-wasting-disease.blogspot.com/2011/01/generation-of-new-form-of-human-prpsc.html


Wednesday, April 06, 2011

Presence and Seeding Activity of Pathological Prion Protein (PrPTSE) in Skeletal Muscles of White-Tailed Deer Infected with Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2011/04/presence-and-seeding-activity-of.html


Wednesday, January 5, 2011

ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011

Prions

David W. Colby1,* and Stanley B. Prusiner1,2

http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html



Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.


Second threat

snip...


http://www.neuroprion.org/en/np-neuroprion.html


http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html


http://prionpathy.blogspot.com/



Rural and Regional Affairs and Transport References Committee

The possible impacts and consequences for public health, trade and agriculture of the Governments decision to relax import restrictions on beef Final report June 2010

2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novohas arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf



CREUTZFELDT JAKOB DISEASE

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencep.../03/mad-cow-atypical-cjd-prion-tse-cases.html




In 2010. we reported 15 new case affected by a condition similar to that previously described for clinical and histopathological features as well as for the character*istics of the abnormal prion protein, but these cases included not only patients who were homozygous Val at codon 129 of the PrP gene like the previous ones but also cases that were homozygous Met (Met/Met) and heterozygous (Met/Val) (Zou et al. 2010a). Because the 129 Met/Met and Metl/Val cases PrPDis presented less and disimilar senitivity to protease digestion, the condition was renamed variably protease-sensitive prionopathy or VPSPr.


Currently, a total of 30 cases of VPSPr have been publihed (Head et al. 2009, 2010; Jansen et al. 20I0; Rodriguez*-Martinez et al. 2010; Zou et al. 2010a). These 30 cases are not equally distributed among the three 129 genotypes: 19 are Val/Val, 8 are Met/Val, and 3 are Met/Met. These findings prompt three considerations. ...



http://transmissiblespongiformencep.../variably-protease-sensitive-prionopathy.html






exposure and accumulation. threshold from sub-clinical to clinical disease, load, these are questions that still cannot be answered. to many i.e. route, source, titre of infectivity, genetics, ....:SHOCKED: what a mess




TSS


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## ridgewalker (Jun 24, 2008)

That is very in depth information. It says more about infection from eating diseased cattle and sheep than from consuming venison. I would not consider eating ground brain material as the mice did nor would I eat those mice that had been intentionally contaminated. The test demonstrated that the particular prion used in that test could be used to spread disease in mice if they were fed ground brain material. That particular prion was not CWD. I have never heard of a hunter eating ground brain material at any rate.

Mad cow disease and scrapies have been around for a long time. The origin of those disease has been speculated about but it has not been determined. A case could be made for not eating European livestock or their products but I fail to see the specific parallel to CWD. Yes, it is a prion disease but that is as far as it goes (according to present knowledge).

The mutations of mrsa and flu (to name two) cause me far more concern than CWD because they actually kill many people across our country.


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## terry (Sep 13, 2002)

ridgewalker said:


> That is very in depth information. It says more about infection from eating diseased cattle and sheep than from consuming venison. I would not consider eating ground brain material as the mice did nor would I eat those mice that had been intentionally contaminated. The test demonstrated that the particular prion used in that test could be used to spread disease in mice if they were fed ground brain material. That particular prion was not CWD. I have never heard of a hunter eating ground brain material at any rate.





somebody is eating them ;


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what about muscle and fat tissue, cwd, consumption, and accumulation there from ???




Prions in Skeletal Muscles of Deer with Chronic Wasting Disease

Rachel C. Angers1,*, Shawn R. Browning1,*?, Tanya S. Seward2, Christina J. Sigurdson4,?, Michael W. Miller5, Edward A. Hoover4 and Glenn C. Telling1,2,3,§ + Author Affiliations

Abstract

The emergence of chronic wasting disease (CWD) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic potential of CWD. Because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.

Received for publication 21 November 2005. Accepted for publication 13 January 2006.

http://www.sciencemag.org/cgi/content/abstract/sci;311/5764/1117



Journal of Virology, September 2009, p. 9608-9610, Vol. 83, No. 18 0022-538X/09/$08.00+0 doi:10.1128/JVI.01127-09 Copyright © 2009, American Society for Microbiology. All Rights Reserved.

Prion Infectivity in Fat of Deer with Chronic Wasting Disease

Brent Race,# Kimberly Meade-White,# Richard Race, and Bruce Chesebro* Rocky Mountain Laboratories, 903 South 4th Street, Hamilton, Montana 59840

Received 2 June 2009/ Accepted 24 June 2009

ABSTRACT Top ABSTRACT TEXT REFERENCES

Chronic wasting disease (CWD) is a neurodegenerative prion disease of cervids. Some animal prion diseases, such as bovine spongiform encephalopathy, can infect humans; however, human susceptibility to CWD is unknown. In ruminants, prion infectivity is found in central nervous system and lymphoid tissues, with smaller amounts in intestine and muscle. In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.

snip...

The highest risk of human contact with CWD might be through exposure to high-titer CNS tissue through accidental skin cuts or corneal contact at the time of harvest and butchering. However, the likelihood of a human consuming fat infected with a low titer of the CWD agent is much higher. It is impossible to remove all the fat present within muscle tissue, and fat consumption is inevitable when eating meat. Of additional concern is the fact that meat from an individual deer harvested by a hunter is typically consumed over multiple meals by the same group of people. These individuals would thus have multiple exposures to the CWD agent over time, which might increase the chance for transfer of infection.

In the Rocky Mountain region of North America, wild deer are subject to predation by wolves, coyotes, bears, and mountain lions. Although canines such as wolves and coyotes are not known to be susceptible to prion diseases, felines definitely are susceptible to BSE (9) and might also be infected by the CWD agent. Deer infected with the CWD agent are more likely to be killed by predators such as mountain lions (11). Peripheral tissues, including lymph nodes, muscle, and fat, which harbor prion infectivity are more accessible for consumption than CNS tissue, which has the highest level of infectivity late in disease. Therefore, infectivity in these peripheral tissues may be important in potential cross-species CWD transmissions in the wild.

The present finding of CWD infectivity in deer fat tissue raises the possibility that prion infectivity might also be found in fat tissue of other infected ruminants, such as sheep and cattle, whose fat and muscle tissues are more widely distributed in both the human and domestic-animal food chains. Although the infectivity in fat tissues is low compared to that in the CNS, there may be significant differences among species and between prion strains. Two fat samples from BSE agent-infected cattle were reported to be negative by bioassay in nontransgenic RIII mice (3, 6). However, RIII mice are 10,000-fold-less sensitive to BSE agent infection than transgenic mice expressing bovine PrP (4). It would be prudent to carry out additional infectivity assays on fat from BSE agent-infected cattle and scrapie agent-infected sheep using appropriate transgenic mice or homologous species to determine the risk from these sources.

http://jvi.asm.org/cgi/content/full/83/18/9608



Detection of CWD prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission

Nicholas J. Haley1, Candace K. Mathiason1, Scott Carver1, Mark Zabel1, Glenn C. Telling2, and Edward A. Hoover1,*

1 Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, USA

2 Department of Molecular Biology and Genetics, University of Kentucky, Lexington, Kentucky, USA

* Corresponding author. Mailing address: Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, 80523. Phone: (970)491-7587, Fax: (970)491-0523. Email: [email protected]

ABSTRACT

Efficient horizontal transmission is a signature trait of chronic wasting disease (CWD) in cervids. Infectious prions shed into excreta appear to play a key role in this facile transmission, as has been demonstrated by bioassay in cervid and transgenic species and serial protein misfolding cyclic amplification (sPMCA). However, the source(s) of infectious prions in these body fluids have yet to be identified. In the present study, we analyzed tissues proximate to saliva, urine, and feces production by sPMCA in an attempt to elucidate this unique aspect of CWD pathogenesis. Oropharyngeal, urogenital, and gastrointestinal tissues, along with blood and obex from CWD-exposed cervids (comprising 27 animals and >350 individual samples) were analyzed and scored based on apparent relative CWD burden. PrPCWD-generating activity was detected in a range of tissues, and was highest in salivary gland, urinary bladder, and the distal intestinal tract. In the same assays, blood from the same animals and unseeded normal brain homogenate controls (n= 116 of 117) remained negative. PrP-converting activity in peripheral tissues varied from 10-11 to 100 - fold that found in brain of the same animal. Deer with highest levels of PrPCWD amplification in the brain had higher and more widely disseminated prion amplification in excretory tissues. Interestingly, PrPCWD was not demonstrable by conventional western blotting in these excretory tissues, suggesting low prion burden or the presence of protease-sensitive infectious prions destroyed by harsh proteolytic treatments. These findings offer unique insights into the transmission of CWD in particular, and prion infection and trafficking overall.


http://jvi.asm.org/cgi/content/abstract/JVI.00425-11v1




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





[/QUOTE]Mad cow disease and scrapies have been around for a long time. The origin of those disease has been speculated about but it has not been determined. A case could be made for not eating European livestock or their products but I fail to see the specific parallel to CWD. Yes, it is a prion disease but that is as far as it goes (according to present knowledge).[/QUOTE]




you are blinded by something then, not to see some sort of parallel to CWD, with the rest of the TSE family. in my opinion all CWD or BSE is, is some strain of sheep scrapie in deer and elk and and some strain of sheep scrapie in cattle. in fact the holding pens at Fort Collins was infected with scrapie, that infected the deer, some of which most likely escaped. 

see ;




2003 The Pathological Protein 


The Fort Collins facility became a CWD death trap. Between 1970 and 1981, 90 percent of the deer that stayed more than two years died from the disease or had to be euthanized. In 1980 the scourge emerged outside Colorado, at the Sybille Research Unit in southeastern Wyoming, 120 miles northwest of Fort Collins. The two facilities had exchanged deer for breeding purposes, thus indicating that the disease was infectious--even to a different species: soon the elk at the facilities contracted the disease. (Deer and elk both belong to the cervid family.)

For years, researchers thought CWD resulted from nutritional deficiencies, poisoning, or stress from confinement. But in 1977 Elizabeth S. Williams, studying for her doctorate at Colorado State University, discovered that this view was mistaken. When Williams looked at brain slices from infected animals, she saw that the tissue was full of microscopic holes. "I happened to be taking a course in neuropathology and had studied a lot of brain lesions," she recalls. The holes were unmistakably like scrapie, the sheep sickness that was the first documented spongiform encephalopathy.

In fact, CWD appears to have originated from scrapie. Richard E. Race of the National Institutes of Health Rocky Mountain Laboratories in Hamilton, Mont., conducted test tube studies that revealed no distinction between the malformed PrP of scrapie sheep and CWD cervids. Consistent with this discovery, Amir Hamir of the U.S. Department of Agriculture's National Animal Disease Center in Ames, Iowa, found no difference in the appearance of brain samples from elk with CWD and elk experimentally infected with scrapie. (BSE also probably arose from scrapie, after cows ate feed derived from infected sheep.)


snip...


By 1985 veterinarians discovered CWD in free-ranging deer and elk, generally within about 30 miles of the two wildlife facilities. Whether the disease originated in the wild and spread to the captives, or vice versa, is not known. The two populations had plenty of time to mingle. Especially during mating season, wild cervids nosed up to captives through the chain-link fences. Incubating deer could also have escaped or been released.

Both facilities tried hard to eradicate CWD. The Sybille center killed all the deer and elk in the affected area and waited a year to introduce new animals; four years later deer and elk started coming down with CWD. The Fort Collins facility acted more aggressively. Officials first killed off all the resident deer and elk; then they turned several inches of soil and repeatedly sprayed structures and pastures with swimming-pool chlorine, which readily wipes out bacteria and viruses. After waiting a year, they brought in 12 elk calves, but a few years afterward two of those elk contracted CWD.


snip...


Philip Yam is Scientific American's news editor. This article is adapted from his book, The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, published in June.



http://www.thepathologicalprotein.com/


also, see ;


1989 

IN CONFIDENCE


Perceptions of unconventional slow virus diseases of animals in the USA



Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or about that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. Whether they were scrapie infected sheep or not is unclear. 


http://collections.europarchive.org...www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf




http://wildlife.state.co.us/NR/rdonlyres/C82EB818-90C6-4D85-897E-9CE279546CCB/0/JWDEpiCWD.pdf




CHRONIC WASTING DISEASE EXPOSURE RISKS FROM COLORADO DIVISION OF WILDLIFE RESEARCH ACTIVITIES


http://wildlife.state.co.us/NR/rdon...FB-AEF8-1AFCF4D00C27/0/research_movements.pdf





[/QUOTE]The mutations of mrsa and flu (to name two) cause me far more concern than CWD because they actually kill many people across our country.[/QUOTE]



so, i suppose sticking ones head in the sand is the answer :help:




CJD9/10022

October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

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

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

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

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

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

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



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



snip...

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

snip...

R. BRADLEY


http://collections.europarchive.org...einquiry.gov.uk/files/yb/1990/09/23001001.pdf



however, i will agree, that MRSA is some nasty stuff. damn near killed me in 2001. 8 weeks vancomycin straight to the heart via long line PIC. 



http://staphmrsa.blogspot.com/




tss


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## trout (Jan 17, 2000)

Thanks for the information.


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