Pathologist dies of suspected Creutzfeldt-Jakob (Mad cow) disease
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Image of brain tissue in a case of variant Creutzfeldt-Jakob disease. CDC photo credit: Sherif Zaki; MD; PhD; Wun-Ju Shieh; MD; PhD; MPH
Creutzfeldt-Jakob disease (mad cow) research pathologist Antonio Ruiz Villaescusa died Sat., March 28, from the disease. Colleagues suspect he may have contracted the disease from exposure to infected human tissue, according to the Barcelona Reporter newspaper.
Ruiz headed the department of pathology at the University Hospital in Madrid and was studying whether the disease is passed on to people who have been exposed to infected tissue.
The head of the pathology department at the Fundación Alcorcón, Dr. Radish, will perform an autopsy to clarify the cause of death and the final results will be announced in about a month, according to the Spanish news site.
Ruiz was recognized internationally for his study in the fields of neuropathology and anatomopatología, and devoted much of his professional life to the study of human transmissible spongiform encephalopathy.
Creutzfeldt-Jakob disease is a rare and invariably fatal brain disorder, according to the National Institute of Neurological Disorders and Stroke. There is currently no single diagnostic test for the disease. The only way to confirm a Creutzfeldt-Jakob disease diagnosis is by brain biopsy or autopsy.
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Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)
http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html
Saturday, January 24, 2009
Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock
2008 Annual Report
http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
see full text ;
http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
Greetings,
IT would seem prudent now, with this recent warning, to take the actions that should have been taken long ago with nutritional supplements.
IT would seem prudent now, to issue this new data on the nutritional supplements web site, it seems to be dead.
http://www.cfsan.fda.gov/~dms/supplmnt.html#dsfl
I find this study most important.
some of us have been saying this for years, myself, i made a submission to the BSE Inquiry in England in 1998. At least they listened.
But i thought some of you would be interested in this.
I don't care really what anybody eats, and or, if you want to pour a gallon of urine on yourself, if it makes you feel good, to go for a hunt. whatever turns you on.
BUT, when you have the CDC finally come out with a warning like this after so many years of floundering, better late than never i suppose, but how many were exposed needlessly???
----- Original Message -----
From: "TERRY SINGELTARY" <flounder9@VERIZON.NET>
To: <CJD-L@LISTS.AEGEE.ORG>
Sent: Thursday, March 19, 2009 8:47 PM
Subject: [CJD-L] Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)
10.3201/eid1505.081458 Suggested citation for this article: Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis. 2009 May; [Epub ahead of print]
Chronic Wasting Disease Prions in Elk Antler Velvet
Rachel C. Angers,1 Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O'Rourke, Aru Balachandran, and Glenn C. Telling Author affiliations: University of Kentucky Medical Center, Lexington, Kentucky, USA (R.C. Angers, T.S. Seward, D. Napier, M. Green, G.C. Telling); Colorado State University, Fort Collins, Colorado, USA (E. Hoover, T. Spraker); US Department of Agriculture, Pullman, Washington, USA (K. O'Rourke); and Canadian Food Inspection Agency, Ottawa, Ontario, Canada (A. Balachandran) 1Current affiliation: MRC Laboratory of Molecular Biology, Cambridge, UK.
Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the apical skin layer covering the growing antler (antler velvet) by using CWD-susceptible transgenic mice and protein misfolding cyclic amplification. Our finding of prions in antler velvet of CWD-affected elk suggests that this tissue may play a role in disease transmission among cervids. Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. The fact that CWD prion incubation times in transgenic mice expressing elk prion protein are consistently more rapid raises the possibility that residue 226, the sole primary structural difference between deer and elk prion protein, may be a major determinant of CWD pathogenesis.
snip...
Discussion
The transmission of CWD prions in antler velvet from 2 naturally affected elk to mice in 2 Tg models demonstrates that this tissue contains low, but detectable, amounts of CWD prions. In addition, serial PMCA amplified otherwise undetectable levels of PrPSc in antler velvet. We characterized CWD prion infectivity by end-point titration. The .6 log i.c.ID50/g CWD prion titer estimated by this method contrasts with .9 log i.c.ID50/g titers of mouse-adapted scrapie prions in rodent brains (9) and .7.7.7 log i.c.ID50/g titers of BSE prions estimated by bioassay in transgenic mice (10,11). The linear relationship between dose and incubation time (12) provides an opportunity to estimate the level of prions in materials containing an unknown amount of infectivity. The attack rates of <100% after inoculation with antler velvet preparations from elk 01-0306 and 03-0306 and the failure to transmit disease from the remaining antler velvet samples suggest that CWD prion titers are close to, or at, the end point of the Tg(CerPrP)1536+/. bioassay. Although we are aware of the limitations of comparing levels of prions in tissues from different CWD-affected cervids, we estimate the end point of the CWD prion titration using D92 to be <3.5 log i.c.ID50 units. Other factors could also influence levels of infectivity in the 4 tested samples, e.g., the portion of the antler processed and the age of the antler when harvested. Histologic evaluation indicated that the velvet samples used in these transmission studies came from elk antlers in the early stages of seasonal growth (data not shown). Whether CWD prion titers in antler velvet vary according to the state of antler growth remains to be determined. Whether prion infectivity is derived from nervous system tissue, blood (13), or another component of velvet, is also unclear. Implications for Horizontal CWD Transmission and Human Exposure Our studies indicate that antler velvet represents a previously unrecognized source of CWD prions in the environment. Whereas oral transmission of rodent-adapted scrapie prions is known to be .5 orders of magnitude less efficient than transmission by intracerebral inoculation (14,15), the relative efficiency of oral CWD prion transmission is unknown. Multiple exposures
Page 7 of 17
to low levels of CWD prions in the environment (16,17), as well as increased infectivity when prions are bound to soil minerals (18), are factors that may influence transmission. The appearance of variant Creutzfeldt-Jakob disease in humans exposed to bovine spongiform encephalopathy (BSE) (19,20) and the demonstration of CWD prions in muscle (3) placed the human species barrier to CWD prions at the forefront of public health concerns. Our studies indicate that antler velvet represents an additional source for human exposure to CWD prions. Widely used in traditional Asian medicine to treat a variety of ailments including impotence, arthritis and high blood pressure, antler velvet can be readily purchased in caplet form and its usage has increased worldwide. Fortunately, to date there is no epidemiologic evidence for increased rates of CJD in the CWD-endemic region (Colorado, USA) (21,22). Also reassuring is the inefficient in vitro conversion of human PrP to protease-resistant PrP by CWD (23). Two studies have shown that CWD prions failed to induce disease in Tg mice expressing human PrP (24,25). However, the failure of BSE to be transmitted to Tg mice expressing human prion protein (HuPrP) was cited as early evidence for a BSE transmission barrier in humans (26); subsequent studies demonstrated a strong effect of the codon 129 polymorphism on transmissibility of BSE prions (27). To date, only mice expressing HuPrP with methionine at 129 have been challenged with CWD. In support of the argument that humans might be susceptible to CWD, intracerebral inoculation of squirrel monkeys produced disease after >30 months (28). Prion strain properties are also critical when considering the potential for interspecies transmission. The existence of multiple CWD strains has been suggested by several studies (4,25,29,30), but strain isolation and host range characterization have not been reported. Finally, it is worth considering that if CWD were to cross the species barrier into humans, this transmission source might not be recognized if the disease profile overlapped with one of the forms of sporadic CJD reported in North America.
Possible Role for Residue 226 in CWD Pathogenesis
Previous studies that demonstrated more rapid CWD prion incubation times in Tg mice expressing elk PrP (24,29) than in Tg(CerPrP)1536+/. mice (4) raised the possibility that the single amino acid difference at residue 226 between elk and deer PrP (5) may influence CWD pathogenesis (29). However, when the transmission characteristics of CWD isolates were directly compared in Tg mice expressing differing levels of deer or elk PrP, Tamguney et al.
Page 8 of 17
concluded that CWD incubation times were related solely to the level of PrP transgene expression (25). We compared CWD transmission in Tg(CerPrP-E226)5037+/. and Tg(CerPrP)1536+/. mice, which express PrP at levels .5-fold higher than PrP in wild type mouse brain (Figure 1A), and found that CWD transmission was consistently and substantially more rapid in Tg(CerPrP-E226)5037+/. mice. Our results appear compatible with more efficient CWD prion propagation by elk cellular prion protein (CerPrPC) containing E at residue 226 than by deer CerPrPC containing Q at this position. Consistent with this interpretation, despite 5-fold lower levels of transgene expression in Tg(CerPrP-E226)5029+/. than in Tg(CerPrP)1536+/. mice, mean incubation times of the D92 isolate were equivalent in these 2 lines (Table). Nonetheless, undetected differences in CerPrPC expression, for example in particular cell types, might result in more rapid disease and/or altered pathologic changes. The generation of transgenic mice expressing elk and deer coding sequences using gene replacement strategies would seem to be an excellent approach for resolving this issue. The different responses to CWD in Tg mice also appear to recapitulate aspects of CWD pathogenesis in the natural hosts. Previous limited comparative transmission studies indicated that CWD developed .25% more rapidly in orally challenged elk than deer (31). Although plaques were not detected in brains of CWD-affected elk, florid plaques have been observed in the brains of diseased deer (32,33). Similar differences in pathologic changes were observed in Tg(CerPrP-E226)5037+/. and Tg(CerPrP)1536+/. mice (Figure 4). Structural analyses suggest that residue 226 is located within a region of PrPC proposed to interact with a factor (34), possibly equivalent to the postulated protein X (35). Although mutation of the equivalent residue from Q to lysine (K) in epitope-tagged mouse PrP had no effect on PrPSc formation in transfected chronically infected ScN2A cells, the effects of the Q-to-E substitution were not assessed (36).
Acknowledgments We thank Dongyue Zhuang for excellent technical assistance. This work was supported by grants 2RO1NS040334-04 from the National Institute
http://www.cdc.gov/eid/content/15/5/pdfs/08-1458.pdf
snip...
1998 MY SUBMISSION TO THE BSE INQUIRY ENGLAND
Sender: "Patricia Cantos" <patricia.cantos at bse.org.uk>
To: "Terry S Singeltary Sr. (E-mail)" <flounder at wt.net>
Subject: Your submission to the Inquiry
Date: Fri, 3 Jul 1998 10:10:05 +0100
3 July 1998 Mr Terry S Singeltary Sr. E-Mail: Flounder at wt.net Ref: E2979
Dear Mr Singeltary,
Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments. Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.
As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.
Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at http://www.bse.org.uk. Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it? In the meantime, thank you for you comments. Please do not hesitate to contact me on 0171 261 8332 should you have any queries.
Yours sincerely Patricia Cantos Families Team Leader Attachments TSS
==============
-------- Original Message --------
Subject: re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''
Date: Thu, 01 May 2003 16:04:35 -0400
From: "Marcia G Crosse" <CrosseM at gao.gov>
To: <flounder at wt.net> CC: "Charles W Davenport" <DavenportC at gao.gov>, "Carolyn Feis Korman" <FeisKormanC at gao.gov>, "Martin Gahart" <GahartM at gao.gov>
Mr. Singletary,
We were informed by representatives of Metabolife, Inc. that Metabolife 356 was reformulated to remove bovine complex as an ingredient in the product, approximately September 2001. We did not independently verify the contents of the product.
Sincerely, Marcia Crosse Acting Director Health CarePublic Health and Science Issues U.S. General Accounting Office 441 G Street, N.W. Washington, D.C. 20548
===================
-------- Original Message -------- Subject: Re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA'' Date: Thu, 01 May 2003 15:48:52 -0500 From: "Terry S. Singeltary Sr." <flounder at wt.net> To: Marcia G Crosse <CrosseM at gao.gov> CC: Charles W Davenport <DavenportC at gao.gov>, Carolyn Feis Korman <FeisKormanC at gao.gov>, Martin Gahart <GahartM at gao.gov> References: <seb14599.014 at GAOGWIA1.GAO.GOV>
THANK YOU!
MIRACLES DO HAPPEN! ;-)
now all we need to do is;
snip......
one small step for man, one giant leap for mankind ;-)
however;
''We did not independently verify the contents of the product''
???
TSS
####### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ########
see history of mad cow in a pill ;
Thursday, March 19, 2009
Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)
http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html
Wednesday, March 18, 2009
Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay
http://chronic-wasting-disease.blogspot.com/2009/03/detection-of-cwd-prions-in-urine-and.html
RECALLS AND FIELD CORRECTIONS: FOODS CLASS II
___________________________________
PRODUCT
a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;
b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;
c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;
d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;
e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;
f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9;
CODE
Elk Meats with production dates of December 29, 30, and 31
RECALLING FIRM/MANUFACTURER
Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009.
Manufacturer: Noah's Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing.
REASON
Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD).
VOLUME OF PRODUCT IN COMMERCE
Unknown
DISTRIBUTION
NV, CA, TX, CO, NY, UT, FL, OK
___________________________________
http://www.fda.gov/bbs/topics/ENFORCE/2009/ENF01099.html
Monday, February 09, 2009
Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD
snip...
Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain
Date: August 25, 2007 at 12:42 pm PST
our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.
http://www.jbc.org/
snip...
Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.
snip...
http://www.emboj.org/current.shtml
snip
http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask] Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
snip...
full text ;
http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html
Wednesday, March 18, 2009
Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK
RECALLS AND FIELD CORRECTIONS: FOODS CLASS II
http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html
============================================================
Saturday, January 24, 2009
Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock
2008 Annual Report
http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
see full text ;
http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
I see what you mean by using the url.
There is still so little known about the disease. The prevailing theory is that CJD is caused by prions (proteins). There's also a buzz going around that it is more prevalent than what is reported to the various health agencies. It can be confused with other diseases and people die so quickly (within a year of contractng it) that it may not be diagnosed, unless family members call for an autopsy...
I've been involved in some other forums and have identified this site to have significantly better written content, so it helps make sense to lastly submit!
PS, how do I change the little picture thingy like some people have, I like it but can't figure it out haha
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