Thursday, December 14, 2017

Governor Scott Walkers Apathy Condemns Wisconsin to a lifetime of Chronic Wasting Disease CWD TSE Prion aka Mad Deer Disease

Wisconsin Conservation Congress District Leadership Council Meeting Agenda Central Wisconsin Convention + Expo Center, Rothschild January 5, 2018, 10:00 a.m. January 6, 2018, 8:00 a.m.

*** 7. Deer & Elk Committee—(postponed question)

http://dnr.wi.gov/About/WCC/Documents/Agenda/2018/DLCouncil.1.5.18.pdf

SATURDAY, NOVEMBER 11, 2017 

Wisconsin Confirmation of chronic wasting disease CWD two white-tailed deer hunting ranch Waupaca County another farm in Iowa County being quarantined


TUESDAY, OCTOBER 31, 2017 

Wisconsin Chronic Wasting Disease CWD TSE Prion Update


SUNDAY, OCTOBER 01, 2017 

WISCONSIN CHRONIC WASTING DISEASE CWD SAMPLING UPDATE


THURSDAY, AUGUST 03, 2017

Wisconsin CWD Showing Up in Northern Wisconsin Deer Farms


THURSDAY, SEPTEMBER 29, 2016 

Wisconsin Governor Scott Walker's DNR et al have floundered again on taking any actions on CWD TSE Prion disease, decides to put off now until March 2017

Wisconsin delays CWD response update Advisory committee to gather more input before new plan is presented in March September 29, 2016


Sunday, May 08, 2016 

*** WISCONSIN CHRONIC WASTING DISEASE CWD TSE PRION SPIRALING FURTHER INTO THE ABYSS UPDATE *** 


Wednesday, February 10, 2016
 
*** Wisconsin Two deer that escaped farm had chronic wasting disease CWD ***
 

Friday, January 29, 2016
 
Wisconsin CWD-positive white-tailed deer found on Iowa County farm January 29, 2016
 

Tuesday, January 19, 2016
 
Wisconsin Second CWD-positive deer found in Oneida County 5-year-old buck shot at Three Lakes Trophy Ranch LLC agency received the CWD-positive report on the animal Dec. 29
 
 
Sunday, January 17, 2016
 
*** Wisconsin Captive CWD Lotto Pays Out Again indemnity payment of $298,770 for 228 white-tailed deer killed on farm ***
 

Wednesday, December 16, 2015
 
Wisconsin Chronic wasting disease confirmed in Crawford County buck harvested on private land
 

$298,770 + $465,000 

Friday, December 04, 2015 

Wisconsin CWD-positive white-tailed deer found on Oneida County hunting preserve December 3, 2015 


Thursday, November 19, 2015
 
Wisconsin Eau Claire Co. deer herd two day round of depopulation CWD testing shows 23 positive
 
 
Wednesday, September 16, 2015
 
*** WISCONSIN CAPTIVE CERVID INDUSTRY RUNNING WILD AND ON THE LOOSE RISKING FURTHER SPREAD OF CWD
 
 
Wednesday, March 04, 2015
 
*** Disease sampling results provide current snapshot of CWD in Wisconsin finding 324 positive detections statewide in 2014
 

TEXAS DEER CZAR SENT TO WISCONSIN TO SOLVE CWD CRISIS, WHILE ROME (TEXAS) BURNS
 
Tuesday, August 11, 2015
 
*** Wisconsin doing what it does best, procrastinating about CWD yet again thanks to Governor Walker ***
 
 
TEXAS DEER CZAR SENT TO WISCONSIN TO SOLVE CWD CRISIS, WHILE ROME (TEXAS) BURNS
 
Friday, June 01, 2012
 
*** TEXAS DEER CZAR TO WISCONSIN ASK TO EXPLAIN COMMENTS
 

MONDAY, NOVEMBER 27, 2017 TEXAS CHRONIC WASTING DISEASE CWD TSE PRION MOUNTING 63 CASES CONFIRMED TO DATE NOVEMBER 27, 2017 http://chronic-wasting-disease.blogspot.com/2017/11/texas-chronic-wasting-disease-cwd-tse.html FRIDAY, DECEMBER 01, 2017 TEXAS TAHC CWD TSE PRION SURVEILLANCE AND REPORTING UNDER QUESTION, 14 CAPTIVE CASES CWD DETECTED 2017 SO FAR http://chronic-wasting-disease.blogspot.com/2017/12/texas-tahc-cwd-tse-prion-surveillance.html SATURDAY, DECEMBER 02, 2017 TEXAS TAHC CWD TSE PRION Trace Herds INs and OUTs Summary Minutes of the 399th and 398th Commission Meeting – 8/22/2017 5/9/2017 http://chronic-wasting-disease.blogspot.com/2017/12/texas-tahc-cwd-tse-prion-trace-herds.html WEDNESDAY, OCTOBER 18, 2017 TEXAS Medina County Elk Tests Positive for Chronic Wasting Disease CWD TSE PRION harvested on a high-fenced premises http://chronic-wasting-disease.blogspot.com/2017/10/texas-medina-county-elk-tests-positive.html

TUESDAY, JULY 21, 2015

Texas CWD Medina County Herd Investigation Update July 16, 2015

Five Year Span
• Trace In: 30 facilities, 126 deer
• Trace Forward: 835 deer to 147 facilities
• 96 breeders
• 46 release sites
• 3 DMPs
• 2 International
Trace In Facilities
• Since June 1, 2010 the index herd has received new deer additions from 30 facilities in 30 counties
• Index herd also has a nursing facility that took in fawns from 1 facility in 2010, data is not represented on this map
see map in link...tss
Trace In Facilities
• Since June 1, 2010 the index herd has received new deer additions from 30 facilities in 30 counties
• Index herd also has a nursing facility that took in fawns from 1 facility in 2010, data is not represented on this map
see map in link...tss
Trace Out Facilities
• Counties in which facility owner received deer from index herd since July 1, 2010
• 66 Texas sites, 2 Mexico sites
• Index herd took in fawns from 1 facility in 2010, data is not represented on this map
• All facility owners that have received deer that traced out from the index breeding facility and nursing facility have been sent hold orders
Herd Management Factors for Consideration
• Per literature, there are two primary sources of exposure to CWD for uninfected deer: Infected deer and contaminated environment
• The CWD test positive white-tail deer was a 2 year old, natural born addition - lateral transmission of disease in the index herd is likely
• Incubation period for CWD can be as long as five years. Due to the relatively young age of this herd, prior testing of mortalities may not accurately reflect the true disease prevalence.
• Significant number of animals moved into and out of the index herd may pose a significant risk to both the captive and wild deer populations in Texas.
• Herd prevalence of disease is crucial in assessing risk in all trace forward and trace in herds directly associated with the index herd.
> 5 year investigation span – trace in and trace forward

this is not enough time for the cwd tse prion, with relations to long term infectious exposure to this farm. see;

*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years***

Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3

http://jgv.sgmjournals.org/content/87/12/3737.full
TEXAS CWD CAPTIVE TRACE OUTS

MONDAY, AUGUST 14, 2017
Thursday, June 13, 2013
 
WISCONSIN DEER FARMING Chronic Wasting Disease CWD DATCP
 
 
Saturday, February 04, 2012
 
Wisconsin 16 MONTH age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised
 
 
Monday, January 16, 2012
 
9 GAME FARMS IN WISCONSIN TEST POSITIVE FOR CWD
 
 
see full text and more here ;
 
 
2010 WISCONSIN CAPTIVE DEER ESCAPES
 
There were 26 reported escape incidents so far this year, this amounted to 20 actual confirmed escape incidents because 3 were previously reported, 2 were confirmed as wild deer, and 1 incident was not confirmed. ... snip... C. & D. Captive Cervid and Law Enforcement Update (11:10 AM)- Warden Pete Dunn gave the captive cervid farm update. There were 26 reported escape incidents so far this year, this amounted to 20 actual confirmed escape incidents because 3 were previously reported, 2 were confirmed as wild deer, and 1 incident was not confirmed. Approximately 30% of these escapes were caused by gates being left open and the other 70% resulted from bad fencing or fence related issues. The 20 actual confirmed escape incidents amounted to 77 total animals. 50 of the escaped animals were recovered or killed and 27 were not recovered and remain unaccounted for. Last year the CWD Committee passed a resolution to require double gates, but this has not gone into effect yet. Questions were raised by the committee about double fencing requirements? Pete responded that double fencing has not been practical or accepted by the industry. The DNR has the authority to do fence inspections. ?If a fence fails to pass the inspection the fencing certificate can be revoked and the farmer can be issued a citation. This year three citations and one warning have been issued for escapes. Pete reviewed the reporting requirements for escape incidents that these must be reported within 24 hours. The farmer then has 72 hours to recover the animals or else it will affect the farm’s herd status and ability to move animals. Davin proposed in the 15 year CWD Plan that the DNR take total control and regulatory authority over all deer farm fencing. Larry Gohlke asked Pete about the reliability for reporting escapes? Pete said that the majority of escapes were reported by the farmer, but it is very difficult to determine when an escape actually occurred. Pete said that they are more concerned that an escape is reported and not that it is reported at the exact time that it happened.
 
 
The Wisconsin DNR has issued a report on the results of an audit of the deer farms in their state. This is a very interesting report and sheds light on the operation of these facilities. A couple of interesting findings is that DNR investigators documented the escape of 436 deer into the wild from game farms. These escapes are from approximately 1/3 of the deer facilities in the state. Additionally, several cash transactions were uncovered where the required shipping tags were not used and record keeping ranged from very meticulous to trying to rely on memory. At one facility, investigators found partially burnt records in a trashcan. The complete report can be downloaded at: 


Thursday, February 09, 2012
 
50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE
 
 
how many states have $465,000., and can quarantine and purchase there from, each cwd said infected farm, but how many states can afford this for all the cwd infected cervid game ranch type farms ???
 
Tuesday, December 20, 2011
 
CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) Farm Update DECEMBER 2011
 
The CWD infection rate was nearly 80%, the highest ever in a North American captive herd.
 
RECOMMENDATION: That the Board approve the purchase of 80 acres of land for $465,000 for the Statewide Wildlife Habitat Program in Portage County and approve the restrictions on public use of the site.
 
SUMMARY:
 
 
 
SEE LATEST CWD MAP WISCONSIN AND ILLINOIS
 
Appendix D. Historical distribu􀆟on of CWD in southern Wisconsin and northern Illinois as of June 30, 2016. Squares represent sec􀆟ons in which CWD has been detected.
 
image
 
 
snip...see full text 16 pages here ;
 
 
Appendix D. Historical distribu􀆟on of CWD in southern Wisconsin and northern Illinois as of June 30, 2016. Squares represent sec􀆟ons in which CWD has been detected. see page 16.
 
 
Cumulative CWD Positive Locations of Wild Deer in Wisconsin and Illinois.
 

*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. 


COLORADO THE ORIGIN OF CHRONIC WASTING DISEASE CWD TSE PRION?

*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
IN CONFIDENCE, REPORT OF AN UNCONVENTIONAL SLOW VIRUS DISEASE IN ANIMALS IN THE USA 1989 http://webarchive.nationalarchives.gov.uk/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
ALSO, one of the most, if not the most top TSE Prion God in Science today is Professor Adriano Aguzzi, and he recently commented on just this, on a cwd post on my facebook page August 20 at 1:44pm, quote;

''it pains me to no end to even comtemplate the possibility, but it seems entirely plausible that CWD originated from scientist-made spread of scrapie from sheep to deer in the colorado research facility. If true, a terrible burden for those involved.'' August 20 at 1:44pm ...end

MONDAY, SEPTEMBER 25, 2017

Colorado Chronic Wasting Disease CWD TSE Prion Mandatory Submission of test samples in some areas and zoonosis


TUESDAY, DECEMBER 12, 2017 

*** Chronic Wasting Disease CWD TSE Prion (aka mad deer disease) Update USA December 14, 2017 ***


Patrick Durkin: DNR news readers could actually use PATRICK DURKIN For the State Journal 

Jun 7, 2017

snip...

Results aren’t yet in on the study’s other 13 macaques. The research is led by Stefanie Czub at the Canadian Food Inspection Agency, and funded by the Alberta Prion Research Institute. Macaques are genetically closer to humans than squirrel monkeys, which previously were found susceptible to prions, the rogue protein that triggers CWD.

-- Only 10 percent of deer shot in Wisconsin’s four most-diseased counties – Dane, Iowa, Sauk and Richland – were tested for CWD during 2016’s hunting seasons.

-- Hunters in those counties registered 23,162 whitetails last fall, and provided test-samples from 2,350 to the Department of Natural Resources. Of those tested, 411, or 17.5 percent, carried CWD. The infection rates were: Dane, 7.3 percent (41 cases); Iowa, 25 percent (220 cases); Sauk, 20.4 percent (90 cases); and Richland, 12.8 percent (60 cases).

-- If those numbers indicate the counties’ CWD prevalence, untested venison from about 3,537 diseased deer will be consumed unknowingly by hunters and their families this year.

When asked about such numbers, former Natural Resources Board chairman Dave Clausen of Amery said: “This is one of the most outrageous human susceptibility experiments in history.”


SUNDAY, DECEMBER 10, 2017 

Detection of Prions in Blood of Cervids at the Asymptomatic Stage of Chronic Wasting Disease

The results showed a sensitivity of 100% in animals with very poor body condition that were IHC-positive in both brain and lymph nodes, 96% in asymptomatic deer IHC-positive in brain and lymph nodes and 53% in animals at early stages of infection that were IHC-positive only in lymph nodes. The overall mean diagnostic sensitivity was 79.3% with 100% specificity. These findings show that PMCA might be useful as a blood test for routine, live animal diagnosis of CWD.



CHRONIC WASTING DISEASE CWD TSE PRION ZOONOTIC ZOONOSIS

PRICE OF TSE PRION POKER GOES UP!

2017

Subject: ***CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat

CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat 

Chronic Wasting Disease (CWD) 

Prevention 

If CWD could spread to people, it would most likely be through eating of infected deer and elk. In a 2006-2007 CDC survey of U.S. residents, nearly 20 percent of those surveyed said they had hunted deer or elk and more than two-thirds said they had eaten venison or elk meat. However, to date, no CWD infections have been reported in people. 

Hunters must consider many factors when determining whether to eat meat from deer and elk harvested from areas with CWD, including the level of risk they are willing to accept. Hunters harvesting wild deer and elk from areas with reported CWD should check state wildlife and public health guidance to see whether testing of animals is recommended or required in a given state or region. In areas where CWD is known to be present, CDC recommends that hunters strongly consider having those animals tested before eating the meat. 

Tests for CWD are monitoring tools that some state wildlife officials use to look at the rates of CWD in certain animal populations. Testing may not be available in every state, and states may use these tests in different ways. A negative test result does not guarantee that an individual animal is not infected with CWD, but it does make it considerably less likely and may reduce your risk of exposure to CWD. 

To be as safe as possible and decrease their potential risk of exposure to CWD, hunters should take the following steps when hunting in areas with CWD: 

Do not shoot, handle or eat meat from deer and elk that look sick or are acting strangely or are found dead (road-kill). When field-dressing a deer: Wear latex or rubber gloves when dressing the animal or handling the meat. Minimize how much you handle the organs of the animal, particularly the brain or spinal cord tissues. Do not use household knives or other kitchen utensils for field dressing. Check state wildlife and public health guidance to see whether testing of animals is recommended or required. Recommendations vary by state, but information about testing is available from many state wildlife agencies. Strongly consider having the deer or elk tested for CWD before you eat the meat. If you have your deer or elk commercially processed, consider asking that your animal be processed individually to avoid mixing meat from multiple animals. If your animal tests positive for CWD, do not eat meat from that animal. The U.S. Department of Agriculture’s Animal and Plant Health Inspection Service regulates commercially farmed deer and elk. The agency operates a national CWD herd certification program. As part of the voluntary program, states and individual herd owners agree to meet requirements meant to decrease the risk of CWD in their herds. Privately owned herds that do not participate in the herd certification program may be at increased risk for CWD. 

Page last reviewed: August 17, 2017 Page last updated: August 17, 2017 Content source: Centers for Disease Control and Prevention National Center for Emerging and Zoonotic Infectious Diseases (NCEZID) Division of High-Consequence Pathogens and Pathology (DHCPP) 


 > However, to date, no CWD infections have been reported in people. 

key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry 

LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ 

*** 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).*** 



Molecular Barriers to Zoonotic Transmission of Prions 

*** 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. 


TUESDAY, SEPTEMBER 12, 2017 

CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat 


Prion 2017 Conference Abstracts CWD

 2017 PRION CONFERENCE 

First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress 

Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 

University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen 

This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. 

Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. 

At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. 

PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS 

Subject: PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS VIDEO 

PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS 

*** PRION 2017 CONFERENCE VIDEO 



 TUESDAY, JUNE 13, 2017

PRION 2017 CONFERENCE ABSTRACT 

First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress


TUESDAY, JULY 04, 2017

*** PRION 2017 CONFERENCE ABSTRACTS ON CHRONIC WASTING DISEASE CWD TSE PRION ***


TUESDAY, JUNE 13, 2017

PRION 2017 CONFERENCE ABSTRACT Chronic Wasting Disease in European moose is associated with PrPSc features different from North American CWD


Wednesday, May 24, 2017 

PRION2017 CONFERENCE VIDEO UPDATE 23 – 26 May 2017 Edinburgh UPDATE 1 


SATURDAY, JULY 29, 2017 

Risk Advisory Opinion: Potential Human Health Risks from Chronic Wasting Disease CFIA, PHAC, HC (HPFB and FNIHB), INAC, Parks Canada, ECCC and AAFC 


Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ 

Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations

 In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.


Prions in Skeletal Muscles of Deer with Chronic Wasting Disease 

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.


*** WDA 2016 NEW YORK *** 

 We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. 

 Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders 


Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo Zoonotic Potential of CWD Prions: An Update 

Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6 

1Department of Pathology, 3National Prion Disease Pathology Surveillance Center, 5Department of Neurology, 6National Center for Regenerative Medicine, Case Western Reserve University, Cleveland, OH 44106, USA. 

4Department of Biological Sciences and Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, 

2Encore Health Resources, 1331 Lamar St, Houston, TX 77010 

Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions. 

PRION 2016 TOKYO 

In Conjunction with Asia Pacific Prion Symposium 2016 

PRION 2016 Tokyo 

Prion 2016 


Cervid to human prion transmission 

Kong, Qingzhong 

Case Western Reserve University, Cleveland, OH, United States 

Abstract 

Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that: 

(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; 

(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence; 

(3) Reliable essays can be established to detect CWD infection in humans;and 

(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches. 

Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3. 

Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1. 

Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions. 

Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans. 

Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans. 

Funding Agency Agency National Institute of Health (NIH) 

Institute National Institute of Neurological Disorders and Stroke (NINDS) 

Type Research Project (R01) 

Project # 1R01NS088604-01A1 

Application # 9037884 

Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) 

Program Officer Wong, May 

Project Start 2015-09-30 

Project End 2019-07-31 

Budget Start 2015-09-30 

Budget End 2016-07-31 

Support Year 1 

Fiscal Year 2015 

Total Cost $337,507 

Indirect Cost $118,756 

Institution 

Name Case Western Reserve University 

Department Pathology 

Type Schools of Medicine 

DUNS # 077758407 

City Cleveland 

State OH 

Country United States 

Zip Code 44106 


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 


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 ; 


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 ***


*** 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. 



BSE INQUIRY

*** 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). *** 

*** 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). *** 

*** 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 ;


you can see more evidence here ;


***check out this old document. some interesting stuff about USA and other Countries and concern for CWD TSE Prion. ...terry

Chronic wasting disease AND TISSUES THAT MIGHT CARRY A RISK FOR HUMAN FOOD AND ANIMAL FEED CHAINS REPORT 

https://ec.europa.eu/food/sites/food/files/safety/docs/sci-com_ssc_out324_en.pdf

SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***


Diagnosis and Reporting of Creutzfeldt-Jakob Disease 

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA Diagnosis and Reporting of Creutzfeldt-Jakob Disease 

To the Editor: 

In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally. 

Terry S. Singeltary, Sr Bacliff, Tex 

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. 


TUESDAY, DECEMBER 12, 2017 

Creutzfeldt Jakob Disease CJD National Prion Disease Pathology Surveillance Center Cases Examined to December 14, 2017


TUESDAY, DECEMBER 12, 2017 

Bovine Spongiform Encephalopathy BSE TSE Prion (aka mad cow disease) Report December 14, 2017 2017


TUESDAY, DECEMBER 12, 2017 

SCRAPIE TSE PRION UPDATE USA DECEMBER 14, 2017


TUESDAY, DECEMBER 12, 2017

Chronic Wasting Disease CWD TSE Prion (aka mad deer disease) Update USA December 14, 2017


DECEMBER 14, 2017, 20 YEARS POST DOD MOM HEIDENHAIN VARIANT CREUTZFELDT JAKOB DISEASE HVCJD DECEMBER 14, 1997, JUST MADE A PROMISE TO MOM, AND YOU DON'T BREAK PROMISES WITH YOUR MOM, NEVER FORGET, AND NEVER LET THEM FORGET...TERRY S. SINGELTARY SR.

Terry S. Singeltary Sr.
 

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