Defining and Assessing Analytical Performance Criteria for Transmissible 
Spongiform Encephalopathy–Detecting Amyloid Seeding 
Assays John G. Gray Affiliations Canadian Bovine Spongifirm Encephalopathy 
Reference Laboratory, Canadian Food Inspection Agency, Lethbridge Laboratory, 
Lethbridge, Alberta Correspondence Address correspondence to John G. Gray, 
M.Sc., Canadian Food Inspection Agency, Lethbridge Laboratory, PO Box 640, 
Township Rd. 9-1, Lethbridge, AB, Canada T1J 3Z4. correspondence x John G. Gray 
snip... 
Transmissible spongiform encephalopathies (TSEs) are infectious, fatal 
neurodegenerative diseases that affect production animal health, and thus human 
food safety. Enhanced TSE detection methods mimic the conjectured basis for 
prion replication, in vitro; biological matrices can be tested for prion 
activity via their ability to convert recombinant cellular prion protein (PrP) 
into amyloid fibrils; fluorescent spectra changes of amyloid-binding 
fluorophores in the reaction vessel detect fibril formation. In vitro PrP 
conversion techniques have high analytical sensitivity for prions, comparable 
with that of bioassays, yet no such protocol has gained regulatory approval for 
use in animal TSE surveillance programs. This study describes a timed in vitro 
PrP conversion protocol with accurate, well-defined analytical criteria based on 
probability density and mass functions of TSE+ and TSE− associated thioflavin T 
signal times, a new approach within this field. The prion detection model used 
is elk chronic wasting disease (CWD) in brain tissues. The protocol and 
analytical criteria proved as sensitive for elk CWD as two bioassay models, and 
upward of approximately 1.2 log10 more sensitive than the most sensitive TSE 
rapid test we assessed. Furthermore, we substantiate that timing in vitro PrP 
conversion may be used to titrate TSE infectivity, and, as a result, provide a 
comprehensive extrapolation of analytical sensitivity differences between 
bioassay, TSE rapid tests, and in vitro PrP conversion for elk CWD.
New Assay Offers Improved Detection of Deadly Prion Diseases 08 April 2016 
Elsevier 
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a 
family of rare progressive, neurodegenerative illnesses that affect both humans 
and animals. TSE surveillance is important for public health and food safety 
because TSEs have the potential of crossing from animals to humans, as seen with 
the spread of mad cow disease, or bovine spongiform encephalopathy (BSE). A 
study in The Journal of Molecular Diagnostics describes an advanced assay that 
offers better sensitivity than currently available tests for detecting a prion 
disease affecting elk.
Timed amyloid seeding assay achieves or surpasses sensitivity of currently 
available tests for transmissible spongiform encephalopathies, according to 
report in The Journal of Molecular Diagnostics
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a 
family of rare progressive, neurodegenerative illnesses that affect both humans 
and animals. TSE surveillance is important for public health and food safety 
because TSEs have the potential of crossing from animals to humans, as seen with 
the spread of mad cow disease, or bovine spongiform encephalopathy (BSE). A 
study in The Journal of Molecular Diagnostics describes an advanced assay that 
offers better sensitivity than currently available tests for detecting a prion 
disease affecting elk.
“The significance of TSEs on human health was not entirely realized until 
cases of variant Creutzfeldt-Jakob disease (vCJD) in humans had been discovered 
in the years following the BSE outbreak in the United Kingdom. These vCJD cases 
were associated with consuming meat products contaminated with BSE prions,” 
explained lead investigator Stefanie Czub, DVM, PhD, of the Canadian BSE 
Reference Lab, Canadian Food Inspection Agency, Lethbridge Laboratory, 
Lethbridge, Alberta (Canada). “The result is that many countries have enacted 
TSE surveillance programs, aiming to eradicate livestock-related TSEs.”
Surveillance programs rely on highly sensitive diagnostic methods to detect 
infections early. Addressing the need to define steadfast analytical performance 
criteria for prion amyloid seeding assays (ASAs), researchers developed a method 
to measure prion protein conversion time (from normal cellular form to prion 
form) by a combination of statistical analyses to obtain a prion-detecting ASA 
with a known degree of confidence. They compared the sensitivity of the new 
assay technique, the timed prion seeding assay (tASA), to other currently 
available tests (two bioassays in laboratory rodents and three commercially 
available TSE rapid tests).
The test samples came from elk brains infected experimentally with chronic 
wasting disease (CWD), a prion disease that affects cervids (hoofed ruminant 
mammals in the deer family). The investigators were able to define clear cut-off 
criteria, allowing determination of TSE-positive and TSE-negative states. Unlike 
TSE rapid tests, ASAs also have the potential to detect and measure TSE 
infection in blood, saliva, or urine. This would offer clinical advantages, such 
as the ability to sample blood instead of relying on more invasive tissue biopsy 
and to screen blood donations for contamination.
“We found that the tASA was at least as sensitive as two rodent bioassays 
and up to 16 times more sensitive than three different TSE rapid tests,” noted 
lead author John G. Gray, MS, Canadian BSE Reference Lab, Canadian Food 
Inspection Agency. “This study should further advance ASAs as recognized prion 
detection systems. We believe this methodology represents the future for prion 
diagnostics, especially concerning human health, for example in screening blood 
donations.”
The tASA is an in vitro method that mimics the conjectured mechanism of 
prion propagation in vivo. It is a conversion assay that uses recombinant 
prion-related protein as a substrate and detects conversion via changes in 
fluorescence. The report describes time specifications for the assay to help 
avoid false-positive results (30 hours) or false-negative results in 
weakly-positive samples (48 hours), as well as the number of replications 
necessary for adequate sensitivity (2-12).
“This study represents an important first step for the tASA diagnostic 
protocol to gain regulatory approval for its use in TSE surveillance programs 
targeting CWD in cervids,” commented noted authority Holger Wille, PhD, of the 
University of Alberta Department of Biochemistry and Centre for Prions and 
Protein Folding Diseases in Edmonton, Alberta (Canada). “Additional work will 
also be needed to fine-tune and test tASA for the detection of prions in 
peripheral organs and environmental samples, which represent a substantial unmet 
need to track the spread of CWD prions among North America cervids as well as in 
the environment.”
Prions are abnormal, transmissible pathogenic agents that induce abnormal 
folding of specific, normal cellular proteins. Because these proteins are 
concentrated in brain tissue, brain damage is characteristic of prion diseases. 
Prion diseases generally progress rapidly and are associated with high 
mortality. Prion disease occurs when the normal cellular form of prion-related 
protein converts or conformationally changes to the disease form. Once the 
disease form is introduced, it becomes self-perpetuating as it converts the 
normal form into more of the disease form. CWD is currently spreading throughout 
the U.S. and Canada, is also present in South Korea, and was just diagnosed in a 
first reindeer in Norway. 
Full bibliographic information“Defining and Assessing Analytical 
Performance Criteria for a TSE-Detecting Amyloid Seeding Assay,”; John G. Gray, 
Catherine Graham, Sandor Dudas, Eric Paxman, Ben Vuong, Stefanie Czub; The 
Journal of Molecular Diagnostics, Volume 18, Issue 3 (May 2016) published by 
Elsevier; DOI: http://dx.doi.org/10.1016/j.jmoldx.2016.01.005 
Friday, April 08, 2016 
Arkansas AGFC Chronic Wasting Disease CWD TSE Prion Confirms 23 Additional 
Cases Total At 79 To Date 
this is extremely disturbing as to what is happening with CWD in Arkansas. 
i don’t want to cause alarm, but there is either an incredible source of this 
CWD coming from some where, maybe even multiple sources, but if not, what about 
a new super strain of CWD, one that spreads with incredible ease ??? I do know 
there are more than one strain of CWD in the wild, how many documented to date I 
do not know. but I am just concerned about a potential super strain of cwd tse 
prion that might be much more contagious, and what the possibility of that might 
be??? 
IMPORTANCE CWD prions are contagious among wild and captive cervids in 
North America and in South Korea. We present data linking the amino acid variant 
Q95H in white-tailed deer cellular prion protein (PrPC) to the emergence of a 
novel CWD strain (H95+). We show that, upon infection, deer expressing H95-PrPC 
molecules accumulated a mixture of CWD strains that selectively propagated 
depending on the PRNP genotype of the host in which they were passaged. Our 
study also demonstrates that mice expressing the deer S96-PRNP allele, 
previously shown to be resistant to various cervid prions, are susceptible to 
H95+ CWD prions. The potential for the generation of novel strains raises the 
possibility of an expanded host range for CWD. 
snip...
Our data suggest that white-tailed deer expressing different PrPC allotypes 
can accumulate and transmit CWD strain mixtures. 
 CWD epizootics involve multiple factors, including the contagious nature 
of the agent, host-pathogen interactions, agent strains, and cervid population 
genetics. Our data indicate that CWD strain emergence is modulated by amino acid 
polymorphisms in the cervid PrP. CWD transmission between hosts with different 
PRNP genotypes (65) has the potential to generate and select novel prion 
conformations. Deer expressing H95-PrPC accumulate CWD prions with different 
transmission properties, as exemplified by its ability to infect resistant 
S96-PRNP mice. Finally, our study highlights the importance of characterizing 
the diversity of CWD strains and their potential for interspecies transmission, 
as various mammalian species are susceptible to experimental CWD infection 
(66–69). Although several lines of evidence suggest that humans are resistant to 
CWD prions (70–73), not all CWD strains have been tested for their zoonotic 
potential. Our results demonstrating that H95+ deer CWD prions have transmission 
properties different from those of CWD prions composed of wt-PrP or S96-PrP 
suggest the need for evaluation of the transmissibility of CWD allotypes. 
P35
ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A 
WISCONSIN STRAIN OF CWD
Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of 
Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 
Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary 
Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 
Department of Biological Sciences, University of Alberta, Edmonton AB, Canada 
T6G 2P5
The identification and characterization of prion strains is increasingly 
important for the diagnosis and biological definition of these infectious 
pathogens. Although well-established in scrapie and, more recently, in BSE, 
comparatively little is known about the possibility of prion strains in chronic 
wasting disease (CWD), a disease affecting free ranging and captive cervids, 
primarily in North America. We have identified prion protein variants in the 
white-tailed deer population and demonstrated that Prnp genotype affects the 
susceptibility/disease progression of white-tailed deer to CWD agent. The 
existence of cervid prion protein variants raises the likelihood of distinct CWD 
strains. Small rodent models are a useful means of identifying prion strains. We 
intracerebrally inoculated hamsters with brain homogenates and phosphotungstate 
concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD 
endemic area) and experimentally infected deer of known Prnp genotypes. These 
transmission studies resulted in clinical presentation in primary passage of 
concentrated CWD prions. Subclinical infection was established with the other 
primary passages based on the detection of PrPCWD in the brains of hamsters and 
the successful disease transmission upon second passage. Second and third 
passage data, when compared to transmission studies using different CWD inocula 
(Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin 
white-tailed deer population is different than the strain(s) present in elk, 
mule-deer and white-tailed deer from the western United States endemic 
region.
*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***
Sunday, March 20, 2016 
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer 
and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission 
Thursday, April 07, 2016 
What is the risk of chronic wasting disease being introduced into Great 
Britain? An updated Qualitative Risk Assessment March 2016
Summary and Full text, with my correspondence with EU, Defra, Scottland, 
Welsh et al over the years at the bottom of this link ;
What is the risk of chronic wasting disease being introduced into Great 
Britain? An updated Qualitative Risk Assessment March 2016 
Saturday, April 02, 2016 
TEXAS TAHC BREAKS IT'S SILENCE WITH TWO MORE CASES CWD CAPTIVE DEER 
BRINGING TOTAL TO 10 CAPTIVES REPORTED TO DATE 
Friday, February 26, 2016 
TEXAS Hartley County Mule Deer Tests Positive for Chronic Wasting Disease 
CWD TSE Prion 
Chronic Wasting Disease CWD TSE Prion Roundup USA April 1, 2016 
Saturday, February 6, 2016 
*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No. 
APHIS-2016-0007] Singeltary Submission 
O.05: Transmission of prions to primates after extended silent incubation 
periods: Implications for BSE and scrapie risk assessment in human populations 
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, 
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys 
Atomic Energy Commission; Fontenay-aux-Roses, France 
Prion diseases (PD) are the unique neurodegenerative proteinopathies 
reputed to be transmissible under field conditions since decades. The 
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that 
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the 
absence of obvious (epidemiological or experimental) elements supporting a 
transmission or genetic predispositions, PD, like the other proteinopathies, are 
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD 
summing 80% of human prion cases). Non-human primate models provided the first 
evidences supporting the transmissibiity of human prion strains and the zoonotic 
potential of BSE. Among them, cynomolgus macaques brought major information for 
BSE risk assessment for human health (Chen, 2014), according to their 
phylogenetic proximity to humans and extended lifetime. We used this model to 
assess the zoonotic potential of other animal PD from bovine, ovine and cervid 
origins even after very long silent incubation periods. 
*** We recently observed the direct transmission of a natural classical 
scrapie isolate to macaque after a 10-year silent incubation period, 
***with features similar to some reported for human cases of sporadic CJD, 
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked 
in humanized mice (Cassard, 2014), 
***is the third potentially zoonotic PD (with BSE and L-type BSE), 
***thus questioning the origin of human sporadic cases. 
We will present an updated panorama of our different transmission studies 
and discuss the implications of such extended incubation periods on risk 
assessment of animal PD for human health. 
=============== 
***thus questioning the origin of human sporadic cases*** 
=============== 
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 
*** Title: Transmission of scrapie prions to primate after an extended 
silent incubation period 
Authors 
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire, 
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item 
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item 
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron, 
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item 
Deslys, Jean-Philippe - 
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal 
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015 
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., 
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., 
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 
2015. Transmission of scrapie prions to primate after an extended silent 
incubation period. Scientific Reports. 5:11573. 
Interpretive Summary: The transmissible spongiform encephalopathies (also 
called prion diseases) are fatal neurodegenerative diseases that affect animals 
and humans. The agent of prion diseases is a misfolded form of the prion protein 
that is resistant to breakdown by the host cells. Since all mammals express 
prion protein on the surface of various cells such as neurons, all mammals are, 
in theory, capable of replicating prion diseases. One example of a prion 
disease, bovine spongiform encephalopathy (BSE; also called mad cow disease), 
has been shown to infect cattle, sheep, exotic undulates, cats, non-human 
primates, and humans when the new host is exposed to feeds or foods contaminated 
with the disease agent. The purpose of this study was to test whether non-human 
primates (cynomologous macaque) are susceptible to the agent of sheep scrapie. 
***After an incubation period of approximately 10 years a macaque developed 
progressive clinical signs suggestive of neurologic disease. 
***Upon postmortem examination and microscopic examination of tissues, 
there was a widespread distribution of lesions consistent with a transmissible 
spongiform encephalopathy. 
***This information will have a scientific impact since it is the first 
study that demonstrates the transmission of scrapie to a non-human primate with 
a close genetic relationship to humans. 
***This information is especially useful to regulatory officials and those 
involved with risk assessment of the potential transmission of animal prion 
diseases to humans. 
Technical Abstract: 
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion 
disease that also causes variant Creutzfeldt-Jakob disease in humans. Over the 
past decades, c-BSE's zoonotic potential has been the driving force in 
establishing extensive protective measures for animal and human health. 
***In complement to the recent demonstration that humanized mice are 
susceptible to scrapie, we report here the first observation of direct 
transmission of a natural classical scrapie isolate to a macaque after a 10-year 
incubation period. 
***Neuropathologic examination revealed all of the features of a prion 
disease: spongiform change, neuronal loss, and accumulation of PrPres throughout 
the CNS. 
***This observation strengthens the questioning of the harmlessness of 
scrapie to humans, at a time when protective measures for human and animal 
health are being dismantled and reduced as c-BSE is considered controlled and 
being eradicated.
***Our results underscore the importance of precautionary and protective 
measures and the necessity for long-term experimental transmission studies to 
assess the zoonotic potential of other animal prion strains. 
***Our study demonstrates susceptibility of adult cattle to oral 
transmission of classical BSE. *** 
***our findings suggest that possible transmission risk of H-type BSE to 
sheep and human. *** 
P.86: Estimating the risk of transmission of BSE and scrapie to ruminants 
and humans by protein misfolding cyclic amplification
Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama 
National Institute of Animal Health; Tsukuba, Japan
To assess the risk of the transmission of ruminant prions to ruminants and 
humans at the molecular level, we investigated the ability of abnormal prion 
protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical 
scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to 
proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding 
cyclic amplification (PMCA).
Six rounds of serial PMCA was performed using 10% brain homogenates from 
transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc 
seed from typical and atypical BSE- or typical scrapie-infected brain 
homogenates from native host species. In the conventional PMCA, the conversion 
of PrPC to PrPres was observed only when the species of PrPC source and PrPSc 
seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA 
and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested 
prion strains. On the other hand, human PrPC was converted by PrPSc from typical 
and H-type BSE in this PMCA condition.
Although these results were not compatible with the previous reports 
describing the lack of transmissibility of H-type BSE to ovine and human 
transgenic mice, 
***our findings suggest that possible transmission risk of H-type BSE to 
sheep and human. 
Bioassay will be required to determine whether the PMCA products are 
infectious to these animals.
================
========================================== 
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS 
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE *** 
O18 
Zoonotic Potential of CWD Prions 
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, 
Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve 
University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 
3Encore Health Resources, Houston, Texas, USA 
*** These results indicate that the CWD prion has the potential to infect 
human CNS and peripheral lymphoid tissues and that there might be asymptomatic 
human carriers of CWD infection. 
================== 
***These results indicate that the CWD prion has the potential to infect 
human CNS and peripheral lymphoid tissues and that there might be asymptomatic 
human carriers of CWD infection.*** 
================== 
P.105: RT-QuIC models trans-species prion transmission 
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover 
Prion Research Center; Colorado State University; Fort Collins, CO USA 
Conversely, FSE maintained sufficient BSE characteristics to more 
efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was 
competent for conversion by CWD and fCWD. 
***This insinuates that, at the level of protein:protein interactions, the 
barrier preventing transmission of CWD to humans is less robust than previously 
estimated. 
================ 
***This insinuates that, at the level of protein:protein interactions, the 
barrier preventing transmission of CWD to humans is less robust than previously 
estimated.*** 
================ 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 *** 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014 
*** chronic wasting disease, there was no absolute barrier to conversion of 
the human prion protein. 
*** Furthermore, the form of human PrPres produced in this in vitro assay 
when seeded with CWD, resembles that found in the most common human prion 
disease, namely sCJD of the MM1 subtype. 
*** These results would seem to suggest that CWD does indeed have zoonotic 
potential, at least as judged by the compatibility of CWD prions and their human 
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests 
that if zoonotic CWD occurred, it would most likely effect those of the PRNP 
codon 129-MM genotype and that the PrPres type would be similar to that found in 
the most common subtype of sCJD (MM1).*** 
*** The potential impact of prion diseases on human health was greatly 
magnified by the recognition that interspecies transfer of BSE to humans by beef 
ingestion resulted in vCJD. While changes in animal feed constituents and 
slaughter practices appear to have curtailed vCJD, there is concern that CWD of 
free-ranging deer and elk in the U.S. might also cross the species barrier. 
Thus, consuming venison could be a source of human prion disease. Whether BSE 
and CWD represent interspecies scrapie transfer or are newly arisen prion 
diseases is unknown. Therefore, the possibility of transmission of prion disease 
through other food animals cannot be ruled out. There is evidence that vCJD can 
be transmitted through blood transfusion. There is likely a pool of unknown size 
of asymptomatic individuals infected with vCJD, and there may be asymptomatic 
individuals infected with the CWD equivalent. These circumstances represent a 
potential threat to blood, blood products, and plasma supplies. 
now, let’s see what the authors said about this casual link, personal 
communications years ago. see where it is stated NO STRONG evidence. so, does 
this mean there IS casual evidence ???? “Our conclusion stating that we found no 
strong evidence of CWD transmission to humans” 
From: TSS (216-119-163-189.ipset45.wt.net) 
Subject: CWD aka MAD DEER/ELK TO HUMANS ??? 
Date: September 30, 2002 at 7:06 am PST 
From: "Belay, Ermias" 
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" 
Sent: Monday, September 30, 2002 9:22 AM 
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS 
Dear Sir/Madam, 
In the Archives of Neurology you quoted (the abstract of which was attached 
to your email), we did not say CWD in humans will present like variant CJD. That 
assumption would be wrong. I encourage you to read the whole article and call me 
if you have questions or need more clarification (phone: 404-639-3091). Also, we 
do not claim that "no-one has ever been infected with prion disease from eating 
venison." Our conclusion stating that we found no strong evidence of CWD 
transmission to humans in the article you quoted or in any other forum is 
limited to the patients we investigated. 
Ermias Belay, M.D. Centers for Disease Control and Prevention 
-----Original Message----- 
From: Sent: Sunday, September 29, 2002 10:15 AM 
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV 
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS 
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS 
Thursday, April 03, 2008 
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 
Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ. 
snip... 
*** twenty-seven CJD patients who regularly consumed venison were reported 
to the Surveillance Center***, 
snip... full text ; 
CJD is so rare in people under age 30, one case in a billion (leaving out 
medical mishaps), that four cases under 30 is "very high," says Colorado 
neurologist Bosque. "Then, if you add these other two from Wisconsin [cases in 
the newspaper], six cases of CJD in people associated with venison is very, very 
high." Only now, with Mary Riley, there are at least seven, and possibly eight, 
with Steve, her dining companion. "It's not critical mass that matters," 
however, Belay says. "One case would do it for me." The chance that two people 
who know each other would both contact CJD, like the two Wisconsin sportsmen, is 
so unlikely, experts say, it would happen only once in 140 years. 
Given the incubation period for TSEs in humans, it may require another 
generation to write the final chapter on CWD in Wisconsin. "Does chronic wasting 
disease pass into humans? We'll be able to answer that in 2022," says Race. 
Meanwhile, the state has become part of an immense experiment. 
I urge everyone to watch this video closely...terry 
*** you can see video here and interview with Jeff's Mom, and scientist 
telling you to test everything and potential risk factors for humans *** 
Envt.07: 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free 
Ranging White-Tailed Deer Infected with Chronic Wasting Disease 
***The presence and seeding activity of PrPTSE in skeletal muscle from 
CWD-infected cervids suggests prevention of such tissue in the human diet as a 
precautionary measure for food safety, pending on further clarification of 
whether CWD may be transmissible to humans. 
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. 
Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡, 
Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ snip... 
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. 
***********CJD REPORT 1994 increased risk for consumption of veal and 
venison and lamb*********** 
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL 
REPORT AUGUST 1994 
Consumption of venison and veal was much less widespread among both cases 
and controls. For both of these meats there was evidence of a trend with 
increasing frequency of consumption being associated with increasing risk of 
CJD. (not nvCJD, but sporadic CJD...tss) 
These associations were largely unchanged when attention was restricted to 
pairs with data obtained from relatives. ... 
Table 9 presents the results of an analysis of these data. 
There is STRONG evidence of an association between ‘’regular’’ veal eating 
and risk of CJD (p = .0.01). 
Individuals reported to eat veal on average at least once a year appear to 
be at 13 TIMES THE RISK of individuals who have never eaten veal. 
There is, however, a very wide confidence interval around this estimate. 
There is no strong evidence that eating veal less than once per year is 
associated with increased risk of CJD (p = 0.51). 
The association between venison eating and risk of CJD shows similar 
pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK 
OF CJD (p = 0.04). 
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY 
OF LAMB EATING (p = 0.02). 
The evidence for such an association between beef eating and CJD is weaker 
(p = 0.14). When only controls for whom a relative was interviewed are included, 
this evidence becomes a little STRONGER (p = 0.08). 
snip... 
It was found that when veal was included in the model with another 
exposure, the association between veal and CJD remained statistically 
significant (p = < 0.05 for all exposures), while the other exposures 
ceased to be statistically significant (p = > 0.05). 
snip... 
In conclusion, an analysis of dietary histories revealed statistical 
associations between various meats/animal products and INCREASED RISK OF CJD. 
When some account was taken of possible confounding, the association between 
VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS 
STATISTICALLY. ... 
snip... 
In the study in the USA, a range of foodstuffs were associated with an 
increased risk of CJD, including liver consumption which was associated with an 
apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 
studies in relation to this particular dietary factor, the risk of liver 
consumption became non-significant with an odds ratio of 1.2 (PERSONAL 
COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS) 
snip...see full report ; 
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 
*** These results would seem to suggest that CWD does indeed have zoonotic 
potential, at least as judged by the compatibility of CWD prions and their human 
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests 
that if zoonotic CWD occurred, it would most likely effect those of the PRNP 
codon 129-MM genotype and that the PrPres type would be similar to that found in 
the most common subtype of sCJD (MM1).*** 
O.05: Transmission of prions to primates after extended silent incubation 
periods: Implications for BSE and scrapie risk assessment in human populations 
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, 
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys 
Atomic Energy Commission; Fontenay-aux-Roses, France 
Prion diseases (PD) are the unique neurodegenerative proteinopathies 
reputed to be transmissible under field conditions since decades. The 
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that 
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the 
absence of obvious (epidemiological or experimental) elements supporting a 
transmission or genetic predispositions, PD, like the other proteinopathies, are 
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD 
summing 80% of human prion cases). Non-human primate models provided the first 
evidences supporting the transmissibiity of human prion strains and the zoonotic 
potential of BSE. Among them, cynomolgus macaques brought major information for 
BSE risk assessment for human health (Chen, 2014), according to their 
phylogenetic proximity to humans and extended lifetime. We used this model to 
assess the zoonotic potential of other animal PD from bovine, ovine and cervid 
origins even after very long silent incubation periods. 
*** We recently observed the direct transmission of a natural classical 
scrapie isolate to macaque after a 10-year silent incubation period, 
***with features similar to some reported for human cases of sporadic CJD, 
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked 
in humanized mice (Cassard, 2014), 
***is the third potentially zoonotic PD (with BSE and L-type BSE), 
***thus questioning the origin of human sporadic cases. We will present an 
updated panorama of our different transmission studies and discuss the 
implications of such extended incubation periods on risk assessment of animal PD 
for human health. 
=============== 
***thus questioning the origin of human sporadic cases*** 
=============== 
***our findings suggest that possible transmission risk of H-type BSE to 
sheep and human. Bioassay will be required to determine whether the PMCA 
products are infectious to these animals. 
============== 
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 
Title: Transmission of scrapie prions to primate after an extended silent 
incubation period 
Authors 
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire, 
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item 
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item 
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron, 
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item 
Deslys, Jean-Philippe - 
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal 
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015 
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., 
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., 
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 
2015. Transmission of scrapie prions to primate after an extended silent 
incubation period. Scientific Reports. 5:11573. 
Interpretive Summary: The transmissible spongiform encephalopathies (also 
called prion diseases) are fatal neurodegenerative diseases that affect animals 
and humans. The agent of prion diseases is a misfolded form of the prion protein 
that is resistant to breakdown by the host cells. Since all mammals express 
prion protein on the surface of various cells such as neurons, all mammals are, 
in theory, capable of replicating prion diseases. One example of a prion 
disease, bovine spongiform encephalopathy (BSE; also called mad cow disease), 
has been shown to infect cattle, sheep, exotic undulates, cats, non-human 
primates, and humans when the new host is exposed to feeds or foods contaminated 
with the disease agent. The purpose of this study was to test whether non-human 
primates (cynomologous macaque) are susceptible to the agent of sheep scrapie. 
After an incubation period of approximately 10 years a macaque developed 
progressive clinical signs suggestive of neurologic disease. Upon postmortem 
examination and microscopic examination of tissues, there was a widespread 
distribution of lesions consistent with a transmissible spongiform 
encephalopathy. This information will have a scientific impact since it is the 
first study that demonstrates the transmission of scrapie to a non-human primate 
with a close genetic relationship to humans. This information is especially 
useful to regulatory officials and those involved with risk assessment of the 
potential transmission of animal prion diseases to humans. Technical Abstract: 
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease 
that also causes variant Creutzfeldt-Jakob disease in humans. Over the past 
decades, c-BSE's zoonotic potential has been the driving force in establishing 
extensive protective measures for animal and human health. 
*** In complement to the recent demonstration that humanized mice are 
susceptible to scrapie, we report here the first observation of direct 
transmission of a natural classical scrapie isolate to a macaque after a 10-year 
incubation period. Neuropathologic examination revealed all of the features of a 
prion disease: spongiform change, neuronal loss, and accumulation of PrPres 
throughout the CNS. 
*** This observation strengthens the questioning of the harmlessness of 
scrapie to humans, at a time when protective measures for human and animal 
health are being dismantled and reduced as c-BSE is considered controlled and 
being eradicated. 
*** Our results underscore the importance of precautionary and protective 
measures and the necessity for long-term experimental transmission studies to 
assess the zoonotic potential of other animal prion strains. 
***This information will have a scientific impact since it is the first 
study that demonstrates the transmission of scrapie to a non-human primate with 
a close genetic relationship to humans. This information is especially useful to 
regulatory officials and those involved with risk assessment of the potential 
transmission of animal prion diseases to humans. 
***This observation strengthens the questioning of the harmlessness of 
scrapie to humans, at a time when protective measures for human and animal 
health are being dismantled and reduced as c-BSE is considered controlled and 
being eradicated. Our results underscore the importance of precautionary and 
protective measures and the necessity for long-term experimental transmission 
studies to assess the zoonotic potential of other animal prion strains. 
why do we not want to do TSE transmission studies on chimpanzees $ 
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. 
snip... 
R. BRADLEY 
”The occurrence of CWD must be viewed against the contest of the locations 
in which it occurred. It was an incidental and unwelcome complication of the 
respective wildlife research programmes. Despite it’s subsequent recognition as 
a new disease of cervids, therefore justifying direct investigation, no specific 
research funding was forthcoming. The USDA veiwed it as a wildlife problem and 
consequently not their province!” page 26. 
In Confidence - Perceptions of unconventional slow virus diseases of 
animals in the USA - APRIL-MAY 1989 - G A H Wells 
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to 
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the 
''Independent'' with cattle being incinerated and thought this was a fanatical 
incident to be avoided in the US at all costs. ... 
MAD COW DISEASE HAS BEEN IN THE USA FOR DECADES, AND I BELIEVE IT WAS IN 
THE USA FIRST, PLEASE SEE ; 
Evidence That Transmissible Mink Encephalopathy Results from Feeding 
Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult 
mink on the farm died from TME. 
snip... 
The rancher was a ''dead stock'' feeder using mostly (>95%) downer 
or dead dairy cattle... 
PL1 
Using in vitro prion replication for high sensitive detection of prions and 
prionlike proteins and for understanding mechanisms of transmission. 
Claudio Soto 
Mitchell Center for Alzheimer's diseases and related Brain disorders, 
Department of Neurology, University of Texas Medical School at Houston. 
Prion and prion-like proteins are misfolded protein aggregates with the 
ability to selfpropagate to spread disease between cells, organs and in some 
cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m 
encephalopathies (TSEs), prions are mostly composed by a misfolded form of the 
prion protein (PrPSc), which propagates by transmitting its misfolding to the 
normal prion protein (PrPC). The availability of a procedure to replicate prions 
in the laboratory may be important to study the mechanism of prion and 
prion-like spreading and to develop high sensitive detection of small quantities 
of misfolded proteins in biological fluids, tissues and environmental samples. 
Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient 
methodology to mimic prion replication in the test tube. PMCA is a platform 
technology that may enable amplification of any prion-like misfolded protein 
aggregating through a seeding/nucleation process. In TSEs, PMCA is able to 
detect the equivalent of one single molecule of infectious PrPSc and propagate 
prions that maintain high infectivity, strain properties and species 
specificity. Using PMCA we have been able to detect PrPSc in blood and urine of 
experimentally infected animals and humans affected by vCJD with high 
sensitivity and specificity. Recently, we have expanded the principles of PMCA 
to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in 
Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to 
study the utility of this technology to detect Aβ and α-syn aggregates in 
samples of CSF and blood from patients affected by these diseases. 
========================= 
***Recently, we have been using PMCA to study the role of environmental 
prion contamination on the horizontal spreading of TSEs. These experiments have 
focused on the study of the interaction of prions with plants and 
environmentally relevant surfaces. Our results show that plants (both leaves and 
roots) bind tightly to prions present in brain extracts and excreta (urine and 
feces) and retain even small quantities of PrPSc for long periods of time. 
Strikingly, ingestion of prioncontaminated leaves and roots produced disease 
with a 100% attack rate and an incubation period not substantially longer than 
feeding animals directly with scrapie brain homogenate. Furthermore, plants can 
uptake prions from contaminated soil and transport them to different parts of 
the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety 
of environmentally relevant surfaces, including stones, wood, metals, plastic, 
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion 
disease when these materials were directly injected into the brain of animals 
and strikingly when the contaminated surfaces were just placed in the animal 
cage. These findings demonstrate that environmental materials can efficiently 
bind infectious prions and act as carriers of infectivity, suggesting that they 
may play an important role in the horizontal transmission of the disease. 
======================== 
Since its invention 13 years ago, PMCA has helped to answer fundamental 
questions of prion propagation and has broad applications in research areas 
including the food industry, blood bank safety and human and veterinary disease 
diagnosis. 
see ; 
Wednesday, December 16, 2015 
Objects in contact with classical scrapie sheep act as a reservoir for 
scrapie transmission 
Objects in contact with classical scrapie sheep act as a reservoir for 
scrapie transmission 
Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, 
Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1 
1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge, 
Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency 
Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and 
Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary 
Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School 
of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, 
UK 
Classical scrapie is an environmentally transmissible prion disease of 
sheep and goats. Prions can persist and remain potentially infectious in the 
environment for many years and thus pose a risk of infecting animals after 
re-stocking. In vitro studies using serial protein misfolding cyclic 
amplification (sPMCA) have suggested that objects on a scrapie affected sheep 
farm could contribute to disease transmission. This in vivo study aimed to 
determine the role of field furniture (water troughs, feeding troughs, fencing, 
and other objects that sheep may rub against) used by a scrapie-infected sheep 
flock as a vector for disease transmission to scrapie-free lambs with the prion 
protein genotype VRQ/VRQ, which is associated with high susceptibility to 
classical scrapie. When the field furniture was placed in clean accommodation, 
sheep became infected when exposed to either a water trough (four out of five) 
or to objects used for rubbing (four out of seven). This field furniture had 
been used by the scrapie-infected flock 8 weeks earlier and had previously been 
shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 
23) through exposure to contaminated field furniture placed within pasture not 
used by scrapie-infected sheep for 40 months, even though swabs from this 
furniture tested negative by PMCA. This infection rate decreased (1 out of 12) 
on the same paddock after replacement with clean field furniture. Twelve grazing 
sheep exposed to field furniture not in contact with scrapie-infected sheep for 
18 months remained scrapie free. The findings of this study highlight the role 
of field furniture used by scrapie-infected sheep to act as a reservoir for 
disease re-introduction although infectivity declines considerably if the field 
furniture has not been in contact with scrapie-infected sheep for several 
months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental 
contamination. 
snip... 
Discussion 
Classical scrapie is an environmentally transmissible disease because it 
has been reported in naïve, supposedly previously unexposed sheep placed in 
pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the 
vector for disease transmission is not known, soil is likely to be an important 
reservoir for prions (2) where – based on studies in rodents – prions can adhere 
to minerals as a biologically active form (21) and remain infectious for more 
than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in 
mule deer housed in paddocks used by infected deer 2 years earlier, which was 
assumed to be through foraging and soil consumption (23). 
Our study suggested that the risk of acquiring scrapie infection was 
greater through exposure to contaminated wooden, plastic, and metal surfaces via 
water or food troughs, fencing, and hurdles than through grazing. Drinking from 
a water trough used by the scrapie flock was sufficient to cause infection in 
sheep in a clean building. Exposure to fences and other objects used for rubbing 
also led to infection, which supported the hypothesis that skin may be a vector 
for disease transmission (9). The risk of these objects to cause infection was 
further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid 
tissue after grazing on one of the paddocks, which contained metal hurdles, a 
metal lamb creep and a water trough in contact with the scrapie flock up to 8 
weeks earlier, whereas no infection had been demonstrated previously in sheep 
grazing on this paddock, when equipped with new fencing and field furniture. 
When the contaminated furniture and fencing were removed, the infection rate 
dropped significantly to 8% of 12 sheep, with soil of the paddock as the most 
likely source of infection caused by shedding of prions from the 
scrapie-infected sheep in this paddock up to a week earlier. 
This study also indicated that the level of contamination of field 
furniture sufficient to cause infection was dependent on two factors: stage of 
incubation period and time of last use by scrapie-infected sheep. Drinking from 
a water trough that had been used by scrapie sheep in the predominantly 
pre-clinical phase did not appear to cause infection, whereas infection was 
shown in sheep drinking from the water trough used by scrapie sheep in the later 
stage of the disease. It is possible that contamination occurred through 
shedding of prions in saliva, which may have contaminated the surface of the 
water trough and subsequently the water when it was refilled. Contamination 
appeared to be sufficient to cause infection only if the trough was in contact 
with sheep that included clinical cases. Indeed, there is an increased risk of 
bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) 
based on PrPSc detection by sPMCA. Although ultraviolet light and heat under 
natural conditions do not inactivate prions (26), furniture in contact with the 
scrapie flock, which was assumed to be sufficiently contaminated to cause 
infection, did not act as vector for disease if not used for 18 months, which 
suggest that the weathering process alone was sufficient to inactivate prions. 
PrPSc detection by sPMCA is increasingly used as a surrogate for 
infectivity measurements by bioassay in sheep or mice. In this reported study, 
however, the levels of PrPSc present in the environment were below the limit of 
detection of the sPMCA method, yet were still sufficient to cause infection of 
in-contact animals. In the present study, the outdoor objects were removed from 
the infected flock 8 weeks prior to sampling and were positive by sPMCA at very 
low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive 
reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay 
could not detect PrPSc on any of the objects above the background of the assay. 
False positive reactions with sPMCA at a low frequency associated with de novo 
formation of infectious prions have been reported (27, 28). This is in contrast 
to our previous study where we demonstrated that outdoor objects that had been 
in contact with the scrapie-infected flock up to 20 days prior to sampling 
harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions 
(12)] and was significantly more positive by the assay compared to analogous 
samples from the scrapie-free farm. This discrepancy could be due to the use of 
a different sPMCA substrate between the studies that may alter the efficiency of 
amplification of the environmental PrPSc. In addition, the present study had a 
longer timeframe between the objects being in contact with the infected flock 
and sampling, which may affect the levels of extractable PrPSc. Alternatively, 
there may be potentially patchy contamination of this furniture with PrPSc, 
which may have been missed by swabbing. The failure of sPMCA to detect 
CWD-associated PrP in saliva from clinically affected deer despite confirmation 
of infectivity in saliva-inoculated transgenic mice was associated with as yet 
unidentified inhibitors in saliva (29), and it is possible that the sensitivity 
of sPMCA is affected by other substances in the tested material. In addition, 
sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more 
difficult from furniture exposed to weather, which is supported by the 
observation that PrPSc was detected by sPMCA more frequently in indoor than 
outdoor furniture (12). A recent experimental study has demonstrated that 
repeated cycles of drying and wetting of prion-contaminated soil, equivalent to 
what is expected under natural weathering conditions, could reduce PMCA 
amplification efficiency and extend the incubation period in hamsters inoculated 
with soil samples (30). This seems to apply also to this study even though the 
reduction in infectivity was more dramatic in the sPMCA assays than in the sheep 
model. Sheep were not kept until clinical end-point, which would have enabled us 
to compare incubation periods, but the lack of infection in sheep exposed to 
furniture that had not been in contact with scrapie sheep for a longer time 
period supports the hypothesis that prion degradation and subsequent loss of 
infectivity occurs even under natural conditions. 
In conclusion, the results in the current study indicate that removal of 
furniture that had been in contact with scrapie-infected animals should be 
recommended, particularly since cleaning and decontamination may not effectively 
remove scrapie infectivity (31), even though infectivity declines considerably 
if the pasture and the field furniture have not been in contact with 
scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in 
furniture that was subjected to weathering, even though exposure led to 
infection in sheep, this method may not always be reliable in predicting the 
risk of scrapie infection through environmental contamination. These results 
suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the 
detection of environmentally associated scrapie, and suggest that extremely low 
levels of scrapie contamination are able to cause infection in susceptible sheep 
genotypes. 
Keywords: classical scrapie, prion, transmissible spongiform 
encephalopathy, sheep, field furniture, reservoir, serial protein misfolding 
cyclic amplification 
Wednesday, December 16, 2015 
*** Objects in contact with classical scrapie sheep act as a reservoir for 
scrapie transmission *** 
Circulation of prions within dust on a scrapie affected farm 
Kevin C Gough1, Claire A Baker2, Hugh A Simmons3, Steve A Hawkins3 and Ben 
C Maddison2* 
Abstract 
Prion diseases are fatal neurological disorders that affect humans and 
animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk 
are contagious prion diseases where environmental reservoirs have a direct link 
to the transmission of disease. Using protein misfolding cyclic amplification we 
demonstrate that scrapie PrPSc can be detected within circulating dusts that are 
present on a farm that is naturally contaminated with sheep scrapie. The 
presence of infectious scrapie within airborne dusts may represent a possible 
route of infection and illustrates the difficulties that may be associated with 
the effective decontamination of such scrapie affected premises. 
snip... 
Discussion 
We present biochemical data illustrating the airborne movement of scrapie 
containing material within a contaminated farm environment. We were able to 
detect scrapie PrPSc within extracts from dusts collected over a 70 day period, 
in the absence of any sheep activity. We were also able to detect scrapie PrPSc 
within dusts collected within pasture at 30 m but not at 60 m distance away from 
the scrapie contaminated buildings, suggesting that the chance of contamination 
of pasture by scrapie contaminated dusts decreases with distance from 
contaminated farm buildings. PrPSc amplification by sPMCA has been shown to 
correlate with infectivity and amplified products have been shown to be 
infectious [14,15]. These experiments illustrate the potential for low dose 
scrapie infectivity to be present within such samples. We estimate low ng levels 
of scrapie positive brain equivalent were deposited per m2 over 70 days, in a 
barn previously occupied by sheep affected with scrapie. This movement of dusts 
and the accumulation of low levels of scrapie infectivity within this 
environment may in part explain previous observations where despite stringent 
pen decontamination regimens healthy lambs still became scrapie infected after 
apparent exposure from their environment alone [16]. The presence of sPMCA 
seeding activity and by inference, infectious prions within dusts, and their 
potential for airborne dissemination is highly novel and may have implications 
for the spread of scrapie within infected premises. The low level circulation 
and accumulation of scrapie prion containing dust material within the farm 
environment will likely impede the efficient decontamination of such scrapie 
contaminated buildings unless all possible reservoirs of dust are removed. 
Scrapie containing dusts could possibly infect animals during feeding and 
drinking, and respiratory and conjunctival routes may also be involved. It has 
been demonstrated that scrapie can be efficiently transmitted via the nasal 
route in sheep [17], as is also the case for CWD in both murine models and in 
white tailed deer [18-20]. 
The sources of dust borne prions are unknown but it seems reasonable to 
assume that faecal, urine, skin, parturient material and saliva-derived prions 
may contribute to this mobile environmental reservoir of infectivity. This work 
highlights a possible transmission route for scrapie within the farm 
environment, and this is likely to be paralleled in CWD which shows strong 
similarities with scrapie in terms of prion dissemination and disease 
transmission. The data indicate that the presence of scrapie prions in dust is 
likely to make the control of these diseases a considerable challenge. 
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 
Title: Scrapie transmits to white-tailed deer by the oral route and has a 
molecular profile similar to chronic wasting disease 
Authors 
item Greenlee, Justin item Moore, S - item Smith, Jodi - item Kunkle, 
Robert item West Greenlee, M - 
Submitted to: American College of Veterinary Pathologists Meeting 
Publication Type: Abstract Only Publication Acceptance Date: August 12, 2015 
Publication Date: N/A Technical Abstract: The purpose of this work was to 
determine susceptibility of white-tailed deer (WTD) to the agent of sheep 
scrapie and to compare the resultant PrPSc to that of the original inoculum and 
chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure 
(concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All 
scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected 
in lymphoid tissues at preclinical time points, and deer necropsied after 28 
months post-inoculation had clinical signs, spongiform encephalopathy, and 
widespread distribution of PrPSc in neural and lymphoid tissues. Western 
blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral 
cortex had a profile similar to the original scrapie inoculum, whereas WB of 
brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile 
resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical 
scrapie were further passaged to mice expressing cervid prion protein and 
intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct 
incubation times. Sheep inoculated intranasally with WTD derived scrapie 
developed disease, but only after inoculation with the inoculum that had a 
scrapie-like profile. The WTD study is ongoing, but deer in both inoculation 
groups are positive for PrPSc by rectal mucosal biopsy. In summary, this work 
demonstrates that WTD are susceptible to the agent of scrapie, two distinct 
molecular profiles of PrPSc are present in the tissues of affected deer, and 
inoculum of either profile readily passes to deer. 
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection 
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion 
Research Unit, National Animal Disease Center, USDA-ARS 
Interspecies transmission studies afford the opportunity to better 
understand the potential host range and origins of prion diseases. Previous 
experiments demonstrated that white-tailed deer are susceptible to sheep-derived 
scrapie by intracranial inoculation. The purpose of this study was to determine 
susceptibility of white-tailed deer to scrapie after a natural route of 
exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal 
(1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep 
clinically affected with scrapie. Non-inoculated deer were maintained as 
negative controls. All deer were observed daily for clinical signs. Deer were 
euthanized and necropsied when neurologic disease was evident, and tissues were 
examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and 
western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) 
due to an injury. At that time, examination of obex and lymphoid tissues by IHC 
was positive, but WB of obex and colliculus were negative. Remaining deer 
developed clinical signs of wasting and mental depression and were necropsied 
from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and 
WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal 
and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work 
demonstrates for the first time that white-tailed deer are susceptible to sheep 
scrapie by potential natural routes of inoculation. In-depth analysis of tissues 
will be done to determine similarities between scrapie in deer after 
intracranial and oral/intranasal inoculation and chronic wasting disease 
resulting from similar routes of inoculation. 
see full text ; 
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed 
deer 
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; 
Agricultural Research Service, National Animal Disease Center; Ames, IA USA 
White-tailed deer are susceptible to the agent of sheep scrapie by 
intracerebral inoculation 
snip... 
It is unlikely that CWD will be eradicated from free-ranging cervids, and 
the disease is likely to continue to spread geographically [10]. However, the 
potential that white-tailed deer may be susceptible to sheep scrapie by a 
natural route presents an additional confounding factor to halting the spread of 
CWD. This leads to the additional speculations that 
1) infected deer could serve as a reservoir to infect sheep with scrapie 
offering challenges to scrapie eradication efforts and 
2) CWD spread need not remain geographically confined to current endemic 
areas, but could occur anywhere that sheep with scrapie and susceptible cervids 
cohabitate. 
This work demonstrates for the first time that white-tailed deer are 
susceptible to sheep scrapie by intracerebral inoculation with a high attack 
rate and that the disease that results has similarities to CWD. These 
experiments will be repeated with a more natural route of inoculation to 
determine the likelihood of the potential transmission of sheep scrapie to 
white-tailed deer. If scrapie were to occur in white-tailed deer, results of 
this study indicate that it would be detected as a TSE, but may be difficult to 
differentiate from CWD without in-depth biochemical analysis. 
2012 
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed 
deer 
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; 
Agricultural Research Service, National Animal Disease Center; Ames, IA USA 
snip... 
The results of this study suggest that there are many similarities in the 
manifestation of CWD and scrapie in WTD after IC inoculation including early and 
widespread presence of PrPSc in lymphoid tissues, clinical signs of depression 
and weight loss progressing to wasting, and an incubation time of 21-23 months. 
Moreover, western blots (WB) done on brain material from the obex region have a 
molecular profile similar to CWD and distinct from tissues of the cerebrum or 
the scrapie inoculum. However, results of microscopic and IHC examination 
indicate that there are differences between the lesions expected in CWD and 
those that occur in deer with scrapie: amyloid plaques were not noted in any 
sections of brain examined from these deer and the pattern of immunoreactivity 
by IHC was diffuse rather than plaque-like. 
*** After a natural route of exposure, 100% of WTD were susceptible to 
scrapie. 
Deer developed clinical signs of wasting and mental depression and were 
necropsied from 28 to 33 months PI. Tissues from these deer were positive for 
PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer 
exhibited two different molecular profiles: samples from obex resembled CWD 
whereas those from cerebrum were similar to the original scrapie inoculum. On 
further examination by WB using a panel of antibodies, the tissues from deer 
with scrapie exhibit properties differing from tissues either from sheep with 
scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are 
strongly immunoreactive when probed with mAb P4, however, samples from WTD with 
scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4 
or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly 
immunoreactive and samples from WTD with scrapie are strongly positive. This 
work demonstrates that WTD are highly susceptible to sheep scrapie, but on first 
passage, scrapie in WTD is differentiable from CWD. 
2011 
*** After a natural route of exposure, 100% of white-tailed deer were 
susceptible to scrapie. 
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection 
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion 
Research Unit, National Animal Disease Center, USDA-ARS 
Interspecies transmission studies afford the opportunity to better 
understand the potential host range and origins of prion diseases. Previous 
experiments demonstrated that white-tailed deer are susceptible to sheep-derived 
scrapie by intracranial inoculation. The purpose of this study was to determine 
susceptibility of white-tailed deer to scrapie after a natural route of 
exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal 
(1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep 
clinically affected with scrapie. Non-inoculated deer were maintained as 
negative controls. All deer were observed daily for clinical signs. Deer were 
euthanized and necropsied when neurologic disease was evident, and tissues were 
examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and 
western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) 
due to an injury. At that time, examination of obex and lymphoid tissues by IHC 
was positive, but WB of obex and colliculus were negative. Remaining deer 
developed clinical signs of wasting and mental depression and were necropsied 
from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and 
WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal 
and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work 
demonstrates for the first time that white-tailed deer are susceptible to sheep 
scrapie by potential natural routes of inoculation. In-depth analysis of tissues 
will be done to determine similarities between scrapie in deer after 
intracranial and oral/intranasal inoculation and chronic wasting disease 
resulting from similar routes of inoculation. 
see full text ; 
Monday, November 3, 2014 
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination 
PPo3-22: 
Detection of Environmentally Associated PrPSc on a Farm with Endemic 
Scrapie 
Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh 
Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of 
Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories 
Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University 
of Nottingham; Sutton Bonington, Loughborough UK 
Key words: scrapie, evironmental persistence, sPMCA 
Ovine scrapie shows considerable horizontal transmission, yet the routes of 
transmission and specifically the role of fomites in transmission remain poorly 
defined. Here we present biochemical data demonstrating that on a 
scrapie-affected sheep farm, scrapie prion contamination is widespread. It was 
anticipated at the outset that if prions contaminate the environment that they 
would be there at extremely low levels, as such the most sensitive method 
available for the detection of PrPSc, serial Protein Misfolding Cyclic 
Amplification (sPMCA), was used in this study. We investigated the distribution 
of environmental scrapie prions by applying ovine sPMCA to samples taken from a 
range of surfaces that were accessible to animals and could be collected by use 
of a wetted foam swab. Prion was amplified by sPMCA from a number of these 
environmental swab samples including those taken from metal, plastic and wooden 
surfaces, both in the indoor and outdoor environment. At the time of sampling 
there had been no sheep contact with these areas for at least 20 days prior to 
sampling indicating that prions persist for at least this duration in the 
environment. These data implicate inanimate objects as environmental reservoirs 
of prion infectivity which are likely to contribute to disease transmission. 
Veterinary Pathology Onlinevet.sagepub.com Published online before print 
February 27, 2014, doi: 10.1177/0300985814524798 Veterinary Pathology February 
27, 2014 0300985814524798 
Lesion Profiling and Subcellular Prion Localization of Cervid Chronic 
Wasting Disease in Domestic Cats 
D. M. Seelig1⇑ A. V. Nalls1 M. Flasik2 V. Frank1 S. Eaton2 C. K. Mathiason1 
E. A. Hoover1 1Department of Microbiology, Immunology, and Pathology, Colorado 
State University, Fort Collins, CO, USA 2Department of Biomedical Sciences, 
Colorado State University, Fort Collins, CO, USA D. M. Seelig, University of 
Minnesota, Department of Veterinary Clinical Sciences, Room 339 VetMedCtrS, 
6192A (Campus Delivery Code), 1352 Boyd Ave, St Paul, MN 55108, USA. Email 
address: dseelig@umn.edu 
Abstract 
Chronic wasting disease (CWD) is an efficiently transmitted, fatal, and 
progressive prion disease of cervids with an as yet to be fully clarified host 
range. While outbred domestic cats (Felis catus) have recently been shown to be 
susceptible to experimental CWD infection, the neuropathologic features of the 
infection are lacking. Such information is vital to provide diagnostic power in 
the event of natural interspecies transmission and insights into host and strain 
interactions in interspecies prion infection. Using light microscopy and 
immunohistochemistry, we detail the topographic pattern of neural spongiosis 
(the “lesion profile”) and the distribution of misfolded prion protein in the 
primary and secondary passage of feline CWD (FelCWD). We also evaluated cellular 
and subcellular associations between misfolded prion protein (PrPD) and central 
nervous system neurons and glial cell populations. From these studies, we (1) 
describe the novel neuropathologic profile of FelCWD, which is distinct from 
either cervid CWD or feline spongiform encephalopathy (FSE), and (2) provide 
evidence of serial passage-associated interspecies prion adaptation. In 
addition, we demonstrate through confocal analysis the successful 
co-localization of PrPD with neurons, astrocytes, microglia, lysosomes, and 
synaptophysin, which, in part, implicates each of these in the neuropathology of 
FelCWD. In conclusion, this work illustrates the simultaneous role of both host 
and strain in the development of a unique FelCWD neuropathologic profile and 
that such a profile can be used to discriminate between FelCWD and FSE. 
prion chronic wasting disease immunohistochemistry interspecies cat feline 
spongiform encephalopathy transmissible spongiform encephalopathy adaptation 
species barrier 
Monday, August 8, 2011 Susceptibility of Domestic Cats to CWD Infection 
Oral.29: Susceptibility of Domestic Cats to CWD Infection 
Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. 
Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason† 
Colorado State University; Fort Collins, CO USA†Presenting author; Email: 
ckm@lamar.colostate.edu 
Domestic and non-domestic cats have been shown to be susceptible to one 
prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted 
through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. 
Because domestic and free ranging felids scavenge cervid carcasses, including 
those in CWD affected areas, we evaluated the susceptibility of domestic cats to 
CWD infection experimentally. Groups of n = 5 cats each were inoculated either 
intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 
40–43 months following IC inoculation, two cats developed mild but progressive 
symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors 
and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on 
the brain of one of these animals (vs. two age-matched controls) performed just 
before euthanasia revealed increased ventricular system volume, more prominent 
sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere 
and in cortical grey distributed through the brain, likely representing 
inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles 
were demonstrated in the brains of both animals by immunodetection assays. No 
clinical signs of TSE have been detected in the remaining primary passage cats 
after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) 
of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC 
inoculated cats are demonstrating abnormal behavior including increasing 
aggressiveness, pacing, and hyper responsiveness. 
*** Two of these cats have developed rear limb ataxia. Although the limited 
data from this ongoing study must be considered preliminary, they raise the 
potential for cervid-to-feline transmission in nature. 
AD.63: 
Susceptibility of domestic cats to chronic wasting disease 
Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin 
Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado 
State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN 
USA 
Domestic and nondomestic cats have been shown to be susceptible to feline 
spongiform encephalopathy (FSE), almost certainly caused by consumption of 
bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and 
free-ranging nondomestic felids scavenge cervid carcasses, including those in 
areas affected by chronic wasting disease (CWD), we evaluated the susceptibility 
of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 
cats each were inoculated either intracerebrally (IC) or orally (PO) with 
CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated 
cats developed signs consistent with prion disease, including a stilted gait, 
weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail 
tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from 
these two cats were pooled and inoculated into cohorts of cats by IC, PO, and 
intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted 
CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased 
incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the 
symptomatic cats by western blotting and immunohistochemistry and abnormalities 
were seen in magnetic resonance imaging, including multifocal T2 fluid 
attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size 
increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 
IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns 
consistent with the early stage of feline CWD. 
*** These results demonstrate that CWD can be transmitted and adapted to 
the domestic cat, thus raising the issue of potential cervid-to- feline 
transmission in nature. 
www.landesbioscience.com 
PO-081: Chronic wasting disease in the cat— Similarities to feline 
spongiform encephalopathy (FSE) 
FELINE SPONGIFORM ENCEPHALOPATHY FSE 
Wednesday, October 17, 2012 
Prion Remains Infectious after Passage through Digestive System of American 
Crows (Corvus brachyrhynchos) 
Chronic Wasting Disease Susceptibility of Four North American Rodents 
Chad J. Johnson1*, Jay R. Schneider2, Christopher J. Johnson2, Natalie A. 
Mickelsen2, Julia A. Langenberg3, Philip N. Bochsler4, Delwyn P. Keane4, Daniel 
J. Barr4, and Dennis M. Heisey2 1University of Wisconsin School of Veterinary 
Medicine, Department of Comparative Biosciences, 1656 Linden Drive, Madison WI 
53706, USA 2US Geological Survey, National Wildlife Health Center, 6006 
Schroeder Road, Madison WI 53711, USA 3Wisconsin Department of Natural 
Resources, 101 South Webster Street, Madison WI 53703, USA 4Wisconsin Veterinary 
Diagnostic Lab, 445 Easterday Lane, Madison WI 53706, USA *Corresponding author 
email: cjohnson@svm.vetmed.wisc.edu 
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 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. 
please see ; 
Chronic Wasting Disease CWD TSE Prion Roundup USA April 1, 2016 
*** Needless conflict *** 
Nature 485, 279–280 (17 May 2012) doi:10.1038/485279b 
Published online 16 May 2012 
Terry S. Singeltary Sr. said: 
I kindly wish to submit the following please ; 
"The fact the Texas cow showed up fairly clearly implied the existence of 
other undetected cases," Dr. Paul Brown, former medical director of the National 
Institutes of Health's Laboratory for Central Nervous System Studies and an 
expert on mad cow-like diseases, told United Press International. "The question 
was, 'How many?' and we still can't answer that." Brown, who is preparing a 
scientific paper based on the latest two mad cow cases to estimate the maximum 
number of infected cows that occurred in the United States, said he has 
"absolutely no confidence in USDA tests before one year ago" because of the 
agency's reluctance to retest the Texas cow that initially tested positive. 
USDA officials finally retested the cow and confirmed it was infected seven 
months later, but only at the insistence of the agency's inspector 
general.
"Everything they did on the Texas cow makes everything they did before 2005 
suspect," Brown said.
Thursday, October 22, 2015 
*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk 
mad cow disease USDA and what really happened ***
Thursday, January 14, 2016 
*** EMERGING ANIMAL DISEASES Actions Needed to Better Position USDA to 
Address Future Risks Report to the Chairman, Committee on Energy and Commerce, 
House of Representatives December 2015 GAO-16-132 
GAO
”The occurrence of CWD must be viewed against the contest of the locations 
in which it occurred. It was an incidental and unwelcome complication of the 
respective wildlife research programmes. Despite it’s subsequent recognition as 
a new disease of cervids, therefore justifying direct investigation, no specific 
research funding was forthcoming. The USDA veiwed it as a wildlife problem and 
consequently not their province!” page 26. 
In Confidence - Perceptions of unconventional slow virus diseases of 
animals in the USA - APRIL-MAY 1989 - G A H Wells 
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to 
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the 
''Independent'' with cattle being incinerated and thought this was a fanatical 
incident to be avoided in the US at all costs. ... 
kind regards, terry

 
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