First successful CWD vaccine tested in deer 
 
Posted Jan. 28, 2015 
 
The first successful vaccination of deer against chronic wasting disease is 
reported in the journal Vaccine, (Vaccine 2015;38:726–33), posted online in 
advance of print Dec. 21, 2014.
 
Researchers say the breakthrough may not only protect U.S. livestock 
against CWD but may also shed new light on human diseases suspected of being 
caused by prion infections, such as Creutzfeldt-Jakob disease, kuru, familial 
insomnia, and variably protease-sensitive prionopathy. Some studies also have 
associated prionlike infections with Alzheimer’s disease.
 
“Now that we have found that preventing prion infection is possible in 
animals, it’s likely feasible in humans as well,” said senior study investigator 
and neurologist Thomas Wisniewski, MD, a professor at New York University’s 
Langone Medical Center.
 
Should further vaccine experiments prove successful, Dr. Wisniewski 
believes as little as 10 percent of a cervid herd would need to be inoculated to 
induce herd immunity.
 
Chronic wasting disease is found in wild and captive cervids of North 
America. Scientists fear CWD could possibly spread to livestock in the same 
regions, especially cattle.
 
For the study, five deer were given the vaccine; another six were given a 
placebo. All the deer were exposed to prion-infected brain tissue. They also 
were housed together, engaging in group activities similar to those in the wild. 
Scientists say this kept them in constant exposure to the infectious 
prions.
 
Deer receiving the vaccine were given eight boosters over 11 months until 
key immune antibodies were detectable in blood, saliva, and feces. The deer also 
were monitored daily for signs of illness, and investigators performed biopsies 
of the animals’ tonsils and gut tissue every three months to search for signs of 
CWD infection.
 
Within two years, all the deer given the placebo had developed CWD. Four 
deer given the vaccine took substantially longer to develop infection, while the 
fifth animal was infection-free at the time of the report’s publication.
 
Dr. Wisniewski and his team made the vaccine using Salmonella bacteria to 
mirror the most common mode of natural infection—ingestion of prion-contaminated 
food or feces. The team inserted a prionlike protein into the genome of an 
attenuated Salmonella bacterium, which produced anti-prion antibodies.
 
“Although our anti-prion vaccine experiments have so far been successful on 
mice and deer, we predict that the method and concept could become a widespread 
technique for not only preventing, but potentially treating, many prion 
diseases,” said lead study investigator Fernando Goni, PhD, an associate 
professor at NYU Langone. 
 
 
Mucosal immunization with an attenuated Salmonella vaccine partially 
protects white-tailed deer from chronic wasting disease 
 
Fernando Goñia, Candace K. Mathiasone, Lucia Yimf, Kinlung Wonga, Jeanette 
Hayes-Kluge, Amy Nallse, Daniel Peysera, Veronica Estevezf, Nathaniel Denkerse, 
Jinfeng Xuc, David A. Osbornh, Karl V. Millerh, Robert J. Warrenh, David R. 
Browng, Jose A. Chabalgoityf, Edward A. Hoovere, Thomas Wisniewskia, b, d, , a 
Department of Neurology, New York University School of Medicine, 550 First 
Avenue, New York, NY 10016, United States b Department of Pathology, New York 
University School of Medicine, 550 First Avenue, New York, NY 10016, United 
States c Department of Population Health, New York University School of 
Medicine, 550 First Avenue, New York, NY 10016, United States d Department of 
Psychiatry, New York University School of Medicine, 550 First Avenue, New York, 
NY 10016, United States e College of Veterinary Medicine and Biomedical 
Sciences, Colorado State University, Fort Collins, CO, United States f 
Laboratory for Vaccine Research, Department of Biotechnology, Instituto de 
Higiene, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay 
g Department of Biology and Biochemistry, University of Bath, UK h Warnell 
School of Forestry and Natural Resources, University of Georgia, United States 
Received 20 August 2014, Revised 13 November 2014, Accepted 19 November 2014, 
Available online 21 December 2014 
 
doi:10.1016/j.vaccine.2014.11.035 Get rights and content
 
--------------------------------------------------------------------------------
 
Highlights
 
• We have developed a Salmonella vaccine strain that expresses cervid 
PrP.
 
• The Salmonella vaccine has been tested in white tailed deer.
 
• The vaccine induced a mucosal and systemic response to cervid PrP and 
PrPCWD.
 
• Vaccinated deer had partial protection for chronic wasting disease.
 
• This is the first partially effective vaccine for a prion disease. 
 
 --------------------------------------------------------------------------------
 
Abstract 
 
Prion disease is a unique category of illness, affecting both animals and 
humans, in which the underlying pathogenesis is related to a conformational 
change of a normal, self-protein called PrPC (C for cellular) to a pathological 
and infectious conformer known as PrPSc (Sc for scrapie). Bovine spongiform 
encephalopathy (BSE), a prion disease believed to have arisen from feeding 
cattle with prion contaminated meat and bone meal products, crossed the species 
barrier to infect humans. Chronic wasting disease (CWD) infects large numbers of 
deer and elk, with the potential to infect humans. Currently no prionosis has an 
effective treatment. Previously, we have demonstrated we could prevent 
transmission of prions in a proportion of susceptible mice with a mucosal 
vaccine. In the current study, white-tailed deer were orally inoculated with 
attenuated Salmonella expressing PrP, while control deer were orally inoculated 
with vehicle attenuated Salmonella. Once a mucosal response was established, the 
vaccinated animals were boosted orally and locally by application of polymerized 
recombinant PrP onto the tonsils and rectal mucosa. The vaccinated and control 
animals were then challenged orally with CWD-infected brain homogenate. Three 
years post CWD oral challenge all control deer developed clinical CWD (median 
survival 602 days), while among the vaccinated there was a significant 
prolongation of the incubation period (median survival 909 days; p = 0.012 by 
Weibull regression analysis) and one deer has remained CWD free both clinically 
and by RAMALT and tonsil biopsies. This negative vaccinate has the highest 
titers of IgA in saliva and systemic IgG against PrP. Western blots showed that 
immunoglobulins from this vaccinate react to PrPCWD. We document the first 
partially successful vaccination for a prion disease in a species naturally at 
risk. 
 
--------------------------------------------------------------------------------
 
Graphical abstract Illustrates the study design of the CWD vaccination 
experiment. There were five vaccinated white-tailed deer and 6 vehicle controls. 
Deer were matched for Prnp genotype (95Q/96G/116A/138S/226Q), except two deer in 
both the control and vaccinated group had the most common polymorphism (∼26% of 
deer) that confers partial resistance to CWD infection, codon 96 G/S instead of 
96 G/G.
 
Full-size image 
 
 
Keywords Prion protein; Immunization; Salmonella vaccine strain; Bovine 
spongiform encephalopathy; Chronic wasting disease; White-tailed deer; Mucosal 
vaccination 
 
Corresponding author at: New York University School of Medicine, ERSP, Rm 
802, 450 East 29th Street, New York, NY 10016, United States. Tel.: +1 212 263 
7993; fax: +1 212 263 7528. Copyright © 2014 Elsevier Ltd. All rights reserved. 
 
 
 Sunday, December 21, 2014 
 
Mucosal immunization with an attenuated Salmonella vaccine partially 
protects white-tailed deer from chronic wasting disease 
 
 
Friday, December 19, 2014 
 
Pan-Provincial Vaccine Enterprise Inc. (PREVENT) Conducting a Chronic 
Wasting Disease (CWD) Vaccine Efficacy Trial in Elk 
 
for some reason I am not sure this is long enough for true test results, and 
party for a vaccine that will stop cwd. don’t get me wrong, it’s good news for a 
change. ...kind regards, terry
 
98 | Veterinary Record | January 24, 2015
 
EDITORIAL
 
Scrapie: a particularly persistent pathogen
 
Cristina Acín
 
Resistant prions in the environment have been the sword of Damocles for 
scrapie control and eradication. Attempts to establish which physical and 
chemical agents could be applied to inactivate or moderate scrapie infectivity 
were initiated in the 1960s and 1970s,with the first study of this type focusing 
on the effect of heat treatment in reducing prion infectivity (Hunter and 
Millson 1964). Nowadays, most of the chemical procedures that aim to inactivate 
the prion protein are based on the method developed by Kimberlin and 
collaborators (1983). This procedure consists of treatment with 20,000 parts per 
million free chlorine solution, for a minimum of one hour, of all surfaces that 
need to be sterilised (in laboratories, lambing pens, slaughterhouses, and so 
on). Despite this, veterinarians and farmers may still ask a range of questions, 
such as ‘Is there an official procedure published somewhere?’ and ‘Is there an 
international organisation which recommends and defines the exact method of 
scrapie decontamination that must be applied?’
 
From a European perspective, it is difficult to find a treatment that could 
be applied, especially in relation to the disinfection of surfaces in lambing 
pens of affected flocks. A 999/2001 EU regulation on controlling spongiform 
encephalopathies (European Parliament and Council 2001) did not specify a 
particular decontamination measure to be used when an outbreak of scrapie is 
diagnosed. There is only a brief recommendation in Annex VII concerning the 
control and eradication of transmissible spongiform encephalopathies (TSE 
s).
 
Chapter B of the regulation explains the measures that must be applied if 
new caprine animals are to be introduced to a holding where a scrapie outbreak 
has previously been diagnosed. In that case, the statement indicates that 
caprine animals can be introduced ‘provided that a cleaning and disinfection of 
all animal housing on the premises has been carried out following 
destocking’.
 
Issues around cleaning and disinfection are common in prion prevention 
recommendations, but relevant authorities, veterinarians and farmers may have 
difficulties in finding the specific protocol which applies. The European Food 
and Safety Authority (EFSA ) published a detailed report about the efficacy of 
certain biocides, such as sodium hydroxide, sodium hypochlorite, guanidine and 
even a formulation of copper or iron metal ions in combination with hydrogen 
peroxide, against prions (EFSA 2009). The report was based on scientific 
evidence (Fichet and others 2004, Lemmer and others 2004, Gao and others 2006, 
Solassol and others 2006) but unfortunately the decontamination measures were 
not assessed under outbreak conditions.
 
The EFSA Panel on Biological Hazards recently published its conclusions on 
the scrapie situation in the EU after 10 years of monitoring and control of the 
disease in sheep and goats (EFSA 2014), and one of the most interesting findings 
was the Icelandic experience regarding the effect of disinfection in scrapie 
control. The Icelandic plan consisted of: culling scrapie-affected sheep or the 
whole flock in newly diagnosed outbreaks; deep cleaning and disinfection of 
stables, sheds, barns and equipment with high pressure washing followed by 
cleaning with 500 parts per million of hypochlorite; drying and treatment with 
300 ppm of iodophor; and restocking was not permitted for at least two years. 
Even when all of these measures were implemented, scrapie recurred on several 
farms, indicating that the infectious agent survived for years in the 
environment, even as many as 16 years after restocking (Georgsson and others 
2006).
 
In the rest of the countries considered in the EFSA (2014) report, 
recommendations for disinfection measures were not specifically defined at the 
government level. In the report, the only recommendation that is made for sheep 
is repopulation with sheep with scrapie-resistant genotypes. This reduces the 
risk of scrapie recurrence but it is difficult to know its effect on the 
infection.
 
Until the EFSA was established (in May 2003), scientific opinions about TSE 
s were provided by the Scientific Steering Committee (SSC) of the EC, whose 
advice regarding inactivation procedures focused on treating animal waste at 
high temperatures (150°C for three hours) and high pressure alkaline hydrolysis 
(SSC 2003). At the same time, the TSE Risk Management Subgroup of the Advisory 
Committee on Dangerous Pathogens (ACDP) in the UK published guidance on safe 
working and the prevention of TSE infection. Annex C of the ACDP report 
established that sodium hypochlorite was considered to be effective, but only if 
20,000 ppm of available chlorine was present for at least one hour, which has 
practical limitations such as the release of chlorine gas, corrosion, 
incompatibility with formaldehyde, alcohols and acids, rapid inactivation of its 
active chemicals and the stability of dilutions (ACDP 2009).
 
In an international context, the World Organisation for Animal Health (OIE) 
does not recommend a specific disinfection protocol for prion agents in its 
Terrestrial Code or Manual. Chapter 4.13 of the Terrestrial Code, General 
recommendations on disinfection and disinsection (OIE 2014), focuses on 
foot-and-mouth disease virus, mycobacteria and Bacillus anthracis, but not on 
prion disinfection. Nevertheless, the last update published by the OIE on bovine 
spongiform encephalopathy (OIE 2012) indicates that few effective 
decontamination techniques are available to inactivate the agent on surfaces, 
and recommends the removal of all organic material and the use of sodium 
hydroxide, or a sodium hypochlorite solution containing 2 per cent available 
chlorine, for more than one hour at 20ºC.
 
The World Health Organization outlines guidelines for the control of TSE s, 
and also emphasises the importance of mechanically cleaning surfaces before 
disinfection with sodium hydroxide or sodium hypochlorite for one hour (WHO 
1999).
 
Finally, the relevant agencies in both Canada and the USA suggest that the 
best treatments for surfaces potentially contaminated with prions are sodium 
hydroxide or sodium hypochlorite at 20,000 ppm. This is a 2 per cent solution, 
while most commercial household bleaches contain 5.25 per cent sodium 
hypochlorite. It is therefore recommended to dilute one part 5.25 per cent 
bleach with 1.5 parts water (CDC 2009, Canadian Food Inspection Agency 
2013).
 
So what should we do about disinfection against prions? First, it is 
suggested that a single protocol be created by international authorities to 
homogenise inactivation procedures and enable their application in all 
scrapie-affected countries. Sodium hypochlorite with 20,000 ppm of available 
chlorine seems to be the procedure used in most countries, as noted in a paper 
summarised on p 99 of this issue of Veterinary Record (Hawkins and others 2015). 
But are we totally sure of its effectiveness as a preventive measure in a 
scrapie outbreak? Would an in-depth study of the recurrence of scrapie disease 
be needed?
 
What we can conclude is that, if we want to fight prion diseases, and 
specifically classical scrapie, we must focus on the accuracy of diagnosis, 
monitoring and surveillance; appropriate animal identification and control of 
movements; and, in the end, have homogeneous and suitable protocols to 
decontaminate and disinfect lambing barns, sheds and equipment available to 
veterinarians and farmers. Finally, further investigations into the resistance 
of prion proteins in the diversity of environmental surfaces are required.
 
References
 
snip...
 
98 | Veterinary Record | January 24, 2015
 
 
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination 
 
Steve A. C. Hawkins, MIBiol, Pathology Department1, Hugh A. Simmons, BVSc 
MRCVS, MBA, MA Animal Services Unit1, Kevin C. Gough, BSc, PhD2 and Ben C. 
Maddison, BSc, PhD3 + Author Affiliations
 
1Animal and Plant Health Agency, Woodham Lane, New Haw, Addlestone, Surrey 
KT15 3NB, UK 2School of Veterinary Medicine and Science, The University of 
Nottingham, Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK 3ADAS 
UK, School of Veterinary Medicine and Science, The University of Nottingham, 
Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK E-mail for 
correspondence: ben.maddison@adas.co.uk Abstract Scrapie of sheep/goats and 
chronic wasting disease of deer/elk are contagious prion diseases where 
environmental reservoirs are directly implicated in the transmission of disease. 
In this study, the effectiveness of recommended scrapie farm decontamination 
regimens was evaluated by a sheep bioassay using buildings naturally 
contaminated with scrapie. Pens within a farm building were treated with either 
20,000 parts per million free chorine solution for one hour or were treated with 
the same but were followed by painting and full re-galvanisation or replacement 
of metalwork within the pen. Scrapie susceptible lambs of the PRNP genotype 
VRQ/VRQ were reared within these pens and their scrapie status was monitored by 
recto-anal mucosa-associated lymphoid tissue. All animals became infected over 
an 18-month period, even in the pen that had been subject to the most stringent 
decontamination process. These data suggest that recommended current guidelines 
for the decontamination of farm buildings following outbreaks of scrapie do 
little to reduce the titre of infectious scrapie material and that environmental 
recontamination could also be an issue associated with these premises. 
 
SNIP...
 
Discussion
 
Thorough pressure washing of a pen had no effect on the amount of 
bioavailable scrapie infectivity (pen B). The routine removal of prions from 
surfaces within a laboratory setting is treatment for a minimum of one hour with 
20,000 ppm free chlorine, a method originally based on the use of brain 
macerates from infected rodents to evaluate the effectiveness of decontamination 
(Kimberlin and others 1983). Further studies have also investigated the 
effectiveness of hypochlorite disinfection of metal surfaces to simulate the 
decontamination of surgical devices within a hospital setting. Such treatments 
with hypochlorite solution were able to reduce infectivity by 5.5 logs to lower 
than the sensitivity of the bioassay used (Lemmer and others 2004). Analogous 
treatment of the pen surfaces did not effectively remove the levels of scrapie 
infectivity over that of the control pens, indicating that this method of 
decontamination is not effective within a farm setting. This may be due to the 
high level of biological matrix that is present upon surfaces within the farm 
environment, which may reduce the amount of free chlorine available to 
inactivate any infectious prion. Remarkably 1/5 sheep introduced into pen D had 
also became scrapie positive within nine months, with all animals in this pen 
being RAMALT positive by 18 months of age. Pen D was no further away from the 
control pen (pen A) than any of the other pens within this barn. Localised hot 
spots of infectivity may be present within scrapie-contaminated environments, 
but it is unlikely that pen D area had an amount of scrapie contamination that 
was significantly different than the other areas within this building. 
Similarly, there were no differences in how the biosecurity of pen D was 
maintained, or how this pen was ventilated compared with the other pens. This 
observation, perhaps, indicates the slower kinetics of disease uptake within 
this pen and is consistent with a more thorough prion removal and 
recontamination. These observations may also account for the presence of 
inadvertent scrapie cases within other studies, where despite stringent 
biosecurity, control animals have become scrapie positive during challenge 
studies using barns that also housed scrapie-affected animals (Ryder and others 
2009). The bioassay data indicate that the exposure of the sheep to a farm 
environment after decontamination efforts thought to be effective in removing 
scrapie is sufficient for the animals to become infected with scrapie. The main 
exposure routes within this scenario are likely to be via the oral route, during 
feeding and drinking, and respiratory and conjunctival routes. It has been 
demonstrated that scrapie infectivity can be efficiently transmitted via the 
nasal route in sheep (Hamir and others 2008), as is the case for CWD in both 
murine models and in white-tailed deer (Denkers and others 2010, 2013). 
Recently, it has also been demonstrated that CWD prions presented as dust when 
bound to the soil mineral montmorillonite can be infectious via the nasal route 
(Nichols and others 2013). When considering pens C and D, the actual source of 
the infectious agent in the pens is not known, it is possible that biologically 
relevant levels of prion survive on surfaces during the decontamination regimen 
(pen C). With the use of galvanising and painting (pen D) covering and sealing 
the surface of the pen, it is possible that scrapie material recontaminated the 
pens by the movement of infectious prions contained within dusts originating 
from other parts of the barn that were not decontaminated or from other areas of 
the farm.
 
Given that scrapie prions are widespread on the surfaces of affected farms 
(Maddison and others 2010a), irrespective of the source of the infectious prions 
in the pens, this study clearly highlights the difficulties that are faced with 
the effective removal of environmentally associated scrapie infectivity. This is 
likely to be paralleled in CWD which shows strong similarities to scrapie in 
terms of both the dissemination of prions into the environment and the facile 
mode of disease transmission. These data further contribute to the understanding 
that prion diseases can be highly transmissible between susceptible individuals 
not just by direct contact but through highly stable environmental reservoirs 
that are refractory to decontamination.
 
The presence of these environmentally associated prions in farm buildings 
make the control of these diseases a considerable challenge, especially in 
animal species such as goats where there is lack of genetic resistance to 
scrapie and, therefore, no scope to re-stock farms with animals that are 
resistant to scrapie.
 
Scrapie Sheep Goats Transmissible spongiform encephalopathies (TSE) 
Accepted October 12, 2014. Published Online First 31 October 2014 
 
 
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. 
 
 
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. 
 
 
*** We conclude that TSE infectivity is likely to survive burial for long 
time periods with minimal loss of infectivity and limited movement from the 
original burial site. However PMCA results have shown that there is the 
potential for rainwater to elute TSE related material from soil which could lead 
to the contamination of a wider area. These experiments reinforce the importance 
of risk assessment when disposing of TSE risk materials. 
 
*** The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
 
PRION 2014 CONFERENCE
 
CHRONIC WASTING DISEASE CWD 
 
A FEW FINDINGS ; 
 
Conclusions. To our knowledge, this is the first established experimental 
model of CWD in TgSB3985. We found evidence for co-existence or divergence of 
two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. 
Finally, we observed phenotypic differences between cervid-derived CWD and 
CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway 
to characterize these strains. 
 
We conclude that TSE infectivity is likely to survive burial for long time 
periods with minimal loss of infectivity and limited movement from the original 
burial site. However PMCA results have shown that there is the potential for 
rainwater to elute TSE related material from soil which could lead to the 
contamination of a wider area. These experiments reinforce the importance of 
risk assessment when disposing of TSE risk materials. 
 
The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
 
Our data establish that meadow voles are permissive to CWD via peripheral 
exposure route, suggesting they could serve as an environmental reservoir for 
CWD. Additionally, our data are consistent with the hypothesis that at least two 
strains of CWD circulate in naturally-infected cervid populations and provide 
evidence that meadow voles are a useful tool for CWD strain typing. 
 
Conclusion. CWD prions are shed in saliva and urine of infected deer as 
early as 3 months post infection and throughout the subsequent >1.5 year 
course of infection. In current work we are examining the relationship of 
prionemia to excretion and the impact of excreted prion binding to surfaces and 
particulates in the environment.
 
Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) 
are shed in urine of infected deer as early as 6 months post inoculation and 
throughout the subsequent disease course. Further studies are in progress 
refining the real-time urinary prion assay sensitivity and we are examining more 
closely the excretion time frame, magnitude, and sample variables in 
relationship to inoculation route and prionemia in naturally and experimentally 
CWD-infected cervids.
 
Conclusions. Our results suggested that the odds of infection for CWD is 
likely controlled by areas that congregate deer thus increasing direct 
transmission (deer-to-deer interactions) or indirect transmission 
(deer-to-environment) by sharing or depositing infectious prion proteins in 
these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely 
controlled by separate factors than found in the Midwestern and endemic areas 
for CWD and can assist in performing more efficient surveillance efforts for the 
region.
 
Conclusions. During the pre-symptomatic stage of CWD infection and 
throughout the course of disease deer may be shedding multiple LD50 doses per 
day in their saliva. CWD prion shedding through saliva and excreta may account 
for the unprecedented spread of this prion disease in nature. 
 
see full text and more ; 
 
Monday, June 23, 2014 
 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD 
 
 
 
*** Infectious agent of sheep scrapie may persist in the environment for at 
least 16 years*** 
 
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3 
 
 
New studies on the heat resistance of hamster-adapted scrapie agent: 
Threshold survival after ashing at 600°C suggests an inorganic template of 
replication 
 
 
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel 
Production 
 
 
Detection of protease-resistant cervid prion protein in water from a 
CWD-endemic area 
 
 
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 
Materials and Wastewater During Processing 
 
 
Rapid assessment of bovine spongiform encephalopathy prion inactivation by 
heat treatment in yellow grease produced in the industrial manufacturing process 
of meat and bone meals 
 
 
Sunday, December 21, 2014 
 
Mucosal immunization with an attenuated Salmonella vaccine partially 
protects white-tailed deer from chronic wasting disease 
 
 
Friday, December 19, 2014 
 
Pan-Provincial Vaccine Enterprise Inc. (PREVENT) Conducting a Chronic 
Wasting Disease (CWD) Vaccine Efficacy Trial in Elk 
 
 
Friday, January 16, 2015 
 
Indiana SENATE BILL No. 442 Miller Pete Hunting wildlife Removes exotic 
mammals from the animals that may be propagated or offered for hunting at a 
shooting preserve Makes it a Class C misdemeanor 
 
 
CHRONIC WASTING DISEASE CWD TSE PRION, how much does it pay to find CWD 
$$$
 
CWD, spreading it around...
 
Tuesday, January 06, 2015 
 
APHIS Provides Additional Information on Chronic Wasting Disease (CWD) 
Indemnity Requests January 5, 2015 05:26 PM EST
 
 
when an industry is catering to the public, with products which can risk 
human and animal health, in my opinion, you should have NO property rights. you 
should not be able to hide behind property rights when you are clearly risking 
human and animal health from your product, or the way you handle that product. 
if you are going to raise, grow, produce a product for the consumer, you have an 
obligation NOT to risk the public domain, public property, and or the wild 
animal populations. just my opinion. ... 
 
Wednesday, January 28, 2015 
 
Another new prion disease: relationship with central and peripheral 
amyloidoses 
 
here we go again...
 
 
spontaneous atypical BSE ???
 
if that's the case, then France is having one hell of an epidemic of 
atypical BSE, probably why they stopped testing for BSE, problem solved 
$$$
 
As of December 2011, around 60 atypical BSE cases have currently been 
reported in 13 countries, *** with over one third in France. 
 
 
so 20 cases of atypical BSE in France, compared to the remaining 40 cases 
in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ 
cases per country, besides Frances 20 cases. you cannot explain this away with 
any spontaneous BSe. ...TSS 
 
Sunday, October 5, 2014
 
France stops BSE testing for Mad Cow Disease
 
 
Thursday, July 24, 2014 
 
*** Protocol for further laboratory investigations into the distribution of 
infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical 
BSE investigations 
 
 
Monday, December 1, 2014
 
Germany Bovine Spongiform Encephalopathy BSE CJD TSE Prion disease A Review 
December 1, 2014 
 
 
Wednesday, January 28, 2015 
 
BOVINE HEPARIN POSITION STATEMENT ON THE REINTRODUCTION and POTENTIAL BSE 
TSE PRION RISK FACTORS THEREFROM 
 
 
Thursday, January 29, 2015 
 
Identification of H-type BSE in Portugal 
 
 
Thursday, January 29, 2015 
 
OIE REPORT Bovine spongiform encephalopathy Prion (atypical BSE type H), 
Norway Information received on 29/01/2015
 
 
Tuesday, December 16, 2014 
 
Evidence for zoonotic potential of ovine scrapie prions 
 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves 
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle 
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia 
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, 
Affiliations Contributions Corresponding author Journal name: Nature 
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 
Article tools Citation Reprints Rights & permissions Article metrics 
 
Abstract 
 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant 
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie 
prions remains unknown. Mice genetically engineered to overexpress the human 
prion protein (tgHu) have emerged as highly relevant models for gauging the 
capacity of prions to transmit to humans. These models can propagate human 
prions without any apparent transmission barrier and have been used used to 
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie 
prions transmit to several tgHu mice models with an efficiency comparable to 
that of cattle BSE. The serial transmission of different scrapie isolates in 
these mice led to the propagation of prions that are phenotypically identical to 
those causing sporadic CJD (sCJD) in humans. These results demonstrate that 
scrapie prions have a zoonotic potential and raise new questions about the 
possible link between animal and human prions.
 
Subject terms: Biological sciences• Medical research At a glance
 
 
why do we not want to do TSE transmission studies on chimpanzees $ 
 
5. A positive result from a chimpanzee challenged severly would likely 
create alarm in some circles even if the result could not be interpreted for 
man. I have a view that all these agents could be transmitted provided a large 
enough dose by appropriate routes was given and the animals kept long enough. 
Until the mechanisms of the species barrier are more clearly understood it might 
be best to retain that hypothesis. 
 
snip... 
 
R. BRADLEY 
 
 
1: J Infect Dis 1980 Aug;142(2):205-8 
 
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to 
nonhuman primates. 
 
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. 
 
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep 
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were 
exposed to the infectious agents only by their nonforced consumption of known 
infectious tissues. The asymptomatic incubation period in the one monkey exposed 
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus 
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the 
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. 
Careful physical examination of the buccal cavities of all of the monkeys failed 
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru 
has remained asymptomatic during the 39 months that it has been under 
observation. 
 
snip... 
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie 
by natural feeding to squirrel monkeys that we have reported provides further 
grounds for concern that scrapie-infected meat may occasionally give rise in 
humans to Creutzfeldt-Jakob disease. 
 
PMID: 6997404 
 
 
Recently the question has again been brought up as to whether scrapie is 
transmissible to man. This has followed reports that the disease has been 
transmitted to primates. One particularly lurid speculation (Gajdusek 1977) 
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and 
transmissible encephalopathy of mink are varieties of a single "virus". The U.S. 
Department of Agriculture concluded that it could "no longer justify or permit 
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human 
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is 
emphasised by the finding that some strains of scrapie produce lesions identical 
to the once which characterise the human dementias" 
 
Whether true or not. the hypothesis that these agents might be 
transmissible to man raises two considerations. First, the safety of laboratory 
personnel requires prompt attention. Second, action such as the "scorched meat" 
policy of USDA makes the solution of the acrapie problem urgent if the sheep 
industry is not to suffer grievously. 
 
snip... 
 
76/10.12/4.6 
 
 
Nature. 1972 Mar 10;236(5341):73-4. 
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). 
 
Gibbs CJ Jr, Gajdusek DC. 
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) 
 
C. J. GIBBS jun. & D. C. GAJDUSEK 
 
National Institute of Neurological Diseases and Stroke, National Institutes 
of Health, Bethesda, Maryland 
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey 
(Macaca fascicularis) with an incubation period of more than 5 yr from the time 
of intracerebral inoculation of scrapie-infected mouse brain. The animal 
developed a chronic central nervous system degeneration, with ataxia, tremor and 
myoclonus with associated severe scrapie-like pathology of intensive astroglial 
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of 
grey matter. The strain of scrapie virus used was the eighth passage in Swiss 
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral 
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, 
Berkshire). 
 
 
 
 
 
2001
 
Suspect symptoms 
 
What if you can catch old-fashioned CJD by eating meat from a sheep 
infected with scrapie? 
 
28 Mar 01 
 
Like lambs to the slaughter 
 
31 March 2001 
 
by Debora MacKenzie Magazine issue 2284. 
 
FOUR years ago, Terry Singeltary watched his mother die horribly from a 
degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary 
was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded 
an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
 
Most doctors believe that sCJD is caused by a prion protein deforming by 
chance into a killer. But Singeltary thinks otherwise. He is one of a number of 
campaigners who say that some sCJD, like the variant CJD related to BSE, is 
caused by eating meat from infected animals. Their suspicions have focused on 
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across 
Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight 
to the campaigners' fears. To their complete surprise, the researchers found 
that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by 
some strains of scrapie," says team member Jean-Philippe Deslys of the French 
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, 
south-west of Paris. Hans Kretschmar of the University of Göttingen, who 
coordinates CJD surveillance in Germany, is so concerned by the findings that he 
now wants to trawl back through past sCJD cases to see if any might have been 
caused by eating infected mutton or lamb.
 
Scrapie has been around for centuries and until now there has been no 
evidence that it poses a risk to human health. But if the French finding means 
that scrapie can cause sCJD in people, countries around the world may have 
overlooked a CJD crisis to rival that caused by BSE.
 
Deslys and colleagues were originally studying vCJD, not sCJD. They 
injected the brains of macaque monkeys with brain from BSE cattle, and from 
French and British vCJD patients. The brain damage and clinical symptoms in the 
monkeys were the same for all three. Mice injected with the original sets of 
brain tissue or with infected monkey brain also developed the same 
symptoms.
 
As a control experiment, the team also injected mice with brain tissue from 
people and animals with other prion diseases: a French case of sCJD; a French 
patient who caught sCJD from human-derived growth hormone; sheep with a French 
strain of scrapie; and mice carrying a prion derived from an American scrapie 
strain. As expected, they all affected the brain in a different way from BSE and 
vCJD. But while the American strain of scrapie caused different damage from 
sCJD, the French strain produced exactly the same pathology.
 
"The main evidence that scrapie does not affect humans has been 
epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute 
for Animal Health in Edinburgh, who was a member of the same team as Deslys. 
"You see about the same incidence of the disease everywhere, whether or not 
there are many sheep, and in countries such as New Zealand with no scrapie." In 
the only previous comparisons of sCJD and scrapie in mice, Bruce found they were 
dissimilar.
 
But there are more than 20 strains of scrapie, and six of sCJD. "You would 
not necessarily see a relationship between the two with epidemiology if only 
some strains affect only some people," says Deslys. Bruce is cautious about the 
mouse results, but agrees they require further investigation. Other trials of 
scrapie and sCJD in mice, she says, are in progress.
 
People can have three different genetic variations of the human prion 
protein, and each type of protein can fold up two different ways. Kretschmar has 
found that these six combinations correspond to six clinical types of sCJD: each 
type of normal prion produces a particular pathology when it spontaneously 
deforms to produce sCJD.
 
But if these proteins deform because of infection with a disease-causing 
prion, the relationship between pathology and prion type should be different, as 
it is in vCJD. "If we look at brain samples from sporadic CJD cases and find 
some that do not fit the pattern," says Kretschmar, "that could mean they were 
caused by infection."
 
There are 250 deaths per year from sCJD in the US, and a similar incidence 
elsewhere. Singeltary and other US activists think that some of these people 
died after eating contaminated meat or "nutritional" pills containing dried 
animal brain. Governments will have a hard time facing activists like Singeltary 
if it turns out that some sCJD isn't as spontaneous as doctors have 
insisted.
 
Deslys's work on macaques also provides further proof that the human 
disease vCJD is caused by BSE. And the experiments showed that vCJD is much more 
virulent to primates than BSE, even when injected into the bloodstream rather 
than the brain. This, says Deslys, means that there is an even bigger risk than 
we thought that vCJD can be passed from one patient to another through 
contaminated blood transfusions and surgical instruments.
 
 
Tuesday, December 23, 2014 
 
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED 
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION 
 
 
 
CWD TO HUMANS, AND RISK FACTORS THERE FROM (see latest science) 
 
Tuesday, November 04, 2014 
 
*** Six-year follow-up of a point-source exposure to CWD contaminated 
venison in an Upstate New York community: risk behaviours and health outcomes 
2005–2011 
 
 
 
Friday, December 5, 2014 
 
*** SPECIAL ALERT The OIE recommends strengthening animal disease 
surveillance worldwide OIE 
 
BSE TSE PRION AKA MAD COW DISEASE ? ‘’the silence was deafening’’ ...tss 
 
 
 Comment from Terry Singeltary Sr. This is a Comment on the Animal and 
Plant Health Inspection Service (APHIS) Notice: Agency Information Collection 
Activities; Proposals, Submissions, and Approvals: Bovine Spongiform 
Encephalopathy; Importation of Animals and Animal Products
 
For related information, Open Docket Folder Docket folder icon 
 
--------------------------------------------------------------------------------
 
Show agency attachment(s) AttachmentsView All (0) Empty
 
--------------------------------------------------------------------------------
 
Comment View document:Docket No. APHIS-2014-0107 Bovine Spongiform 
Encephalopathy; Importation of Animals and Animal Products Singeltary Submission 
;
 
I believe that there is more risk to the world from Transmissible 
Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from 
the United States and all of North America, than there is risk coming to the USA 
and North America, from other Countries. I am NOT saying I dont think there is 
any risk for the BSE type TSE prion coming from other Countries, I am just 
saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present 
mad cow risk factors in North America like they are not here? 
 
North America has more strains of TSE prion disease, in more species 
(excluding zoo animals in the early BSE days, and excluding the Feline TSE and 
or Canine TSE, because they dont look, and yes, there has been documented 
evidence and scientific studies, and DEFRA Hound study, that shows the canine 
spongiform encephalopathy is very possible, if it has not already happened, just 
not documented), then any other Country in the world. Mink TME, Deer Elk cervid 
CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type 
BSE cattle, atyical HG type BSE cow (the only cow documented in the world to 
date with this strain), typical sheep goat Scrapie (multiple strains), and the 
atypical Nor-98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical 
Scrapie has spread from coast to coast. sporadic CJD on the rise, with different 
strains mounting, victims becoming younger, with the latest nvCJD human mad cow 
case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL 
CDC. 
 
typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), 
and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical 
Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid 
populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk 
assessments for each country, and then made BSE confirmed countries legal to 
trade mad cow disease, which was all brought forth AFTER that fateful day 
December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats 
the day it all started. once the BSE MRR policy was shoved down every countries 
throat by USDA inc and the OIE, then the legal trading of Scrapie was validated 
to be a legal trading commodity, also shoved through by the USDA inc and the 
OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion 
disease typical and atypical strains, and the BSE TSE Prion aka mad cow type 
disease was thus made a legal trading commodity, like it or not. its all about 
money now folks, trade, to hell with human health with a slow incubating 
disease, that is 100% fatal once clinical, and forget the fact of exposure, 
sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion 
disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its 
all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the 
infamous VPSPr. ...problem solved $$$ 
 
the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing 
but ink on paper. 
 
for this very reason I believe the BSE MRR policy is a total failure, and 
that this policy should be immediately withdrawn, and set back in place the BSE 
GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all 
TSE PRION disease in all species of animals, and that the BSE GBR risk 
assessments be made stronger than before. 
 
lets start with the recent notice that beef from Ireland will be coming to 
America. 
 
Ireland confirmed around 1655 cases of mad cow disease. with the highest 
year confirming about 333 cases in 2002, with numbers of BSE confirmed cases 
dropping from that point on, to a documentation of 1 confirmed case in 2013, to 
date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad 
cow feed ban, and the enforcement of that ban, has drastically reduced the 
number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the 
USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 
2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD 
COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in 
my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow 
disease in the USA, we still have no clue as to the true number of cases of BSE 
mad cow disease in the USA or North America as a whole. ...just saying. 
 
Number of reported cases of bovine spongiform encephalopathy (BSE) in 
farmed cattle worldwide* (excluding the United Kingdom) 
 
Country/Year 
 
snip...please see attached pdf file, with references of breaches in the USA 
triple BSE mad cow firewalls, and recent science on the TSE prion disease. 
...TSS No documents available. AttachmentsView All (1) Empty Docket No. 
APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and 
Animal Products Singeltary Submission View Attachment: 
 
 
Sunday, January 11, 2015 
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of 
Animals and Animal Products Singeltary Submission 
 
 
 
 *** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL 
CDC ***
 
Sunday, November 23, 2014 
 
*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas 
in June 2014 confirmed as USA case NOT European 
 
the patient had resided in Kuwait, Russia and Lebanon. The completed 
investigation did not support the patient's having had extended travel to 
European countries, including the United Kingdom, or travel to Saudi Arabia. The 
specific overseas country where this patient’s infection occurred is less clear 
largely because the investigation did not definitely link him to a country where 
other known vCJD cases likely had been infected. 
 
 
Sunday, December 14, 2014 
 
*** ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD 
strains, TSE prion aka Mad Cow Disease United States of America Update December 
14, 2014 Report
 
 
Wednesday, January 28, 2015 
 
Another new prion disease: relationship with central and peripheral 
amyloidoses here we go again... 
 
 
Thursday, January 15, 2015 
 
41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE 
Prion: Case Report 
 
 
Subject: *** Becky Lockhart 46, Utah’s first female House speaker, dies 
diagnosed with the extremely rare Creutzfeldt-Jakob disease aka mad cow type 
disease
 
what is CJD ? just ask USDA inc., and the OIE, they are still feeding the 
public and the media industry fed junk science that is 30 years old. 
 
why doesn’t some of you try reading the facts, instead of rubber stamping 
everything the USDA inc says.
 
sporadic CJD has now been linked to BSE aka mad cow disease, Scrapie, and 
there is much concern now for CWD and risk factor for humans. 
 
My sincere condolences to the family and friends of the House Speaker Becky 
Lockhart. I am deeply saddened hear this. 
 
with that said, with great respect, I must ask each and every one of you 
Politicians that are so deeply saddened to hear of this needless death of the 
Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am 
seriously going to ask you all this...I have been diplomatic for about 17 years 
and it has got no where. people are still dying. so, are you all stupid or 
what??? how many more need to die ??? how much is global trade of beef and other 
meat products that are not tested for the TSE prion disease, how much and how 
many bodies is this market worth? 
 
Saturday, January 17, 2015 
 
*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed 
with the extremely rare Creutzfeldt-Jakob disease
 
 
 
Thursday, January 22, 2015 
 
Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to 
disease etiology?
 
 
Tuesday, December 30, 2014 
 
TSEAC USA Reason For Recalls Blood products, collected from a donors 
considered to be at increased risk for Creutzfeldt-Jakob Disease (CJD), were 
distributed END OF YEAR REPORT 2014
 
 
who’s kidding whom $$$ i.e. USDA INC AND THE OIE 
 
2014 
 
***Moreover, L-BSE has been transmitted more easily to transgenic mice 
overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE. 
 
***It has been suggested that some sporadic CJD subtypes in humans may 
result from an exposure to the L-BSE agent. 
 
*** Lending support to this hypothesis, pathological and biochemical 
similarities have been observed between L-BSE and an sCJD subtype (MV genotype 
at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and 
another sCJD subtype (MM genotype) [15]. 
 
snip... 
 
 
Monday, October 10, 2011 
 
EFSA Journal 2011 The European Response to BSE: A Success Story 
 
snip... 
 
EFSA and the European Centre for Disease Prevention and Control (ECDC) 
recently delivered a scientific opinion on any possible epidemiological or 
molecular association between TSEs in animals and humans (EFSA Panel on 
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical 
BSE prions as the only TSE agents demonstrated to be zoonotic so far 
 
*** but the possibility that a small proportion of human cases so far 
classified as "sporadic" CJD are of zoonotic origin could not be excluded. 
 
*** Moreover, transmission experiments to non-human primates suggest that 
some TSE agents in addition to Classical BSE prions in cattle (namely L-type 
Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) 
and chronic wasting disease (CWD) agents) might have zoonotic potential. 
 
snip... 
 
 
 
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... 
 
 
Friday, January 30, 2015
 
Scrapie: a particularly persistent pathogen
 
 
 Terry S. Singeltary Sr.