The Prion Institute focuses on the following areas of research excellence: protein folding and misfolding; pathobiology of TSEs; surveillance and control; and TSEs and society.
Applications must be submitted through the Agriculture Funding Consortium website.
ALBERTA ALZHEIMER RESEARCH PROGRAM
The Alberta Prion Research Institute (Prion Institute) and the Alzheimer Society of Alberta and Northwest Territories (ASANT) present the Alberta Alzheimer Research Program (AARP). The AARP allows qualified Alberta investigators to seek funding for research directly related to Alzheimer’s disease in areas related to understanding the fundamental mechanisms of the disease and/or improving the quality of life of those with Alzheimer’s disease. There are two streams for grants: Young Investigator Grants and Regular Research Grants. Young Investigator Grants are available to Alberta researchers who are within five years of their first appointment after completing their research training. These grants are for up to $200,000 with a term of up to three years. Regular Research Grants are available to all Alberta researchers, including young investigators. These grants are for up to $150,000 with a term of up to three years.
Application Deadline: January 16, 2017
Program Guidelines
Funding Declaration
Budget Spreadsheet
EXPLORATIONS
The Explorations Program allows Alberta-based investigators to seek funding for research directly related to prion diseases and prion-like human neurodegenerative diseases and dementias. The research themes for this program are protein folding and misfolding in prion diseases, the pathobiology of transmissible spongiform encephalopathies (TSEs), surveillance and control of prion diseases, TSEs and society, protein folding and misfolding in prion-like human neurodegenerative diseases, and prion-like mechanisms in human neurodegenerative diseases. The Alberta Prion Research Institute offers two tiers of funding for the Explorations competition: grants of up to $200,000 for a maximum of two years and grants of up to $500,000 for a maximum of three years.
Application Deadline: no current competition
SPECIFIED RISK MATERIALS PROGRAM
The Specified Risk Materials Program is intended to allow qualified investigators to seek funding for research directly related to the following areas of specified risk materials (SRM): detection of prions in complex matrices; SRM as feedstock for processes and products; disposition and disposal methods; cost benefit estimates of existing or new disposition and disposal methodologies; risk assessment; risk communication about SRM and risks and benefits of disposition and disposal of SRM; and other. Funding is accessible for all relevant fields of inquiry in the themes as described in the guideline to develop innovative disposition and disposal methods and/or uses of specified risk materials. This competition will support grants of up to $500,000 for a period of up to three years.
Application Deadline: no current competition
RESEARCH TEAM PROGRAM
The Research Team Program allows teams of qualified investigators to seek funding for research directly related to prion diseases and prion-like human neurodegenerative diseases and dementias. The research themes for this program are protein folding and misfolding in prion diseases, the pathobiology of TSEs, surveillance and control of prion diseases, TSEs and society, protein folding and misfolding in prion-like human neurodegenerative diseases and prion-like mechanisms in the pathobiology of prion-like human neurodegenerative diseases. The program is designed to encourage collaboration within Alberta and between Alberta-based investigators and researchers outside of Alberta. The team of researchers must be from at least two different research institutions. The Alberta Prion Research Institute provides up to $750,000 for a team grant over a maximum of three years.
Application Deadline: no current competition
IDeal PROGRAM
The IDeal Program, also known as the Innovation and Delivery Program, was created to encourage collaboration among industry, Alberta universities and research institutions. The Alberta Prion Research Institute invites applications for research and development projects undertaken by Alberta-based private sector organizations or public sector researchers and their private sector partners. A private sector partner may be a company, an industry association or similar body. Direct project costs are shared by private sector partners and the funders. IDeal grants are typically of an applied nature and directed at a specific challenge identified by the private sector partner and the industry. Themes accessible for funding are:
Application Deadline: continuous intake
GUEST SPEAKER SUPPORT PROGRAM
The Guest Speaker Support Program is designed to disseminate knowledge to the Alberta research and science community by offering funding to help bring notable researchers in prion and protein misfolding science to Alberta to present lectures and to participate in panel discussions. These experts assist with planning or implementing collaborative research programs and/or explore and evaluate recent breakthroughs in prion science. Speakers might be involved in research related activities or public studies or both. Funds are provided primarily to offset the costs of travel and accommodation for guest speakers. Support of up to $1,500 is provided for speakers travelling within North America, and up to $2,500 is provided for speakers travelling from outside North America. In some cases, the Alberta Prion Research Institute will consider application for a contribution toward the cost of conducting an associated conference, symposia or workshop.
Application Deadline: continuous intake
EXPANDING HORIZONS PROGRAM
The Expanding Horizons Program is a professional development program for graduate students and postdoctoral fellows who are currently working on prion research. The objective of this program is to provide research trainees with access to mentoring in order to develop skills and knowledge that are applicable both within and outside of the lab. Seminars focus on career development in research as well as other careers they might undertake such as those in industry or policy making. Ensuring trainees are skilled in topics like lab management, networking, communicating, interview skills and grant crafting will benefit their futures as well as the future of prion research.
Applications must be submitted through the Agriculture Funding Consortium website.
ALBERTA ALZHEIMER RESEARCH PROGRAM
The Alberta Prion Research Institute (Prion Institute) and the Alzheimer Society of Alberta and Northwest Territories (ASANT) present the Alberta Alzheimer Research Program (AARP). The AARP allows qualified Alberta investigators to seek funding for research directly related to Alzheimer’s disease in areas related to understanding the fundamental mechanisms of the disease and/or improving the quality of life of those with Alzheimer’s disease. There are two streams for grants: Young Investigator Grants and Regular Research Grants. Young Investigator Grants are available to Alberta researchers who are within five years of their first appointment after completing their research training. These grants are for up to $200,000 with a term of up to three years. Regular Research Grants are available to all Alberta researchers, including young investigators. These grants are for up to $150,000 with a term of up to three years.
Application Deadline: January 16, 2017
Program Guidelines
Funding Declaration
Budget Spreadsheet
EXPLORATIONS
The Explorations Program allows Alberta-based investigators to seek funding for research directly related to prion diseases and prion-like human neurodegenerative diseases and dementias. The research themes for this program are protein folding and misfolding in prion diseases, the pathobiology of transmissible spongiform encephalopathies (TSEs), surveillance and control of prion diseases, TSEs and society, protein folding and misfolding in prion-like human neurodegenerative diseases, and prion-like mechanisms in human neurodegenerative diseases. The Alberta Prion Research Institute offers two tiers of funding for the Explorations competition: grants of up to $200,000 for a maximum of two years and grants of up to $500,000 for a maximum of three years.
Application Deadline: no current competition
SPECIFIED RISK MATERIALS PROGRAM
The Specified Risk Materials Program is intended to allow qualified investigators to seek funding for research directly related to the following areas of specified risk materials (SRM): detection of prions in complex matrices; SRM as feedstock for processes and products; disposition and disposal methods; cost benefit estimates of existing or new disposition and disposal methodologies; risk assessment; risk communication about SRM and risks and benefits of disposition and disposal of SRM; and other. Funding is accessible for all relevant fields of inquiry in the themes as described in the guideline to develop innovative disposition and disposal methods and/or uses of specified risk materials. This competition will support grants of up to $500,000 for a period of up to three years.
Application Deadline: no current competition
RESEARCH TEAM PROGRAM
The Research Team Program allows teams of qualified investigators to seek funding for research directly related to prion diseases and prion-like human neurodegenerative diseases and dementias. The research themes for this program are protein folding and misfolding in prion diseases, the pathobiology of TSEs, surveillance and control of prion diseases, TSEs and society, protein folding and misfolding in prion-like human neurodegenerative diseases and prion-like mechanisms in the pathobiology of prion-like human neurodegenerative diseases. The program is designed to encourage collaboration within Alberta and between Alberta-based investigators and researchers outside of Alberta. The team of researchers must be from at least two different research institutions. The Alberta Prion Research Institute provides up to $750,000 for a team grant over a maximum of three years.
Application Deadline: no current competition
IDeal PROGRAM
The IDeal Program, also known as the Innovation and Delivery Program, was created to encourage collaboration among industry, Alberta universities and research institutions. The Alberta Prion Research Institute invites applications for research and development projects undertaken by Alberta-based private sector organizations or public sector researchers and their private sector partners. A private sector partner may be a company, an industry association or similar body. Direct project costs are shared by private sector partners and the funders. IDeal grants are typically of an applied nature and directed at a specific challenge identified by the private sector partner and the industry. Themes accessible for funding are:
- Protein folding and misfolding in prion diseases;
- The pathobiology of TSEs;
- Surveillance and control of prion diseases;
- Diagnostic technologies;
- Solutions to enhance market access;
- Innovative disposal and/or uses of specified risk material;
- TSEs and society;
- Protein folding and misfolding in prion-like human neurodegenerative diseases; and
- Prion-like mechanisms in human neurodegenerative diseases.
Application Deadline: continuous intake
GUEST SPEAKER SUPPORT PROGRAM
The Guest Speaker Support Program is designed to disseminate knowledge to the Alberta research and science community by offering funding to help bring notable researchers in prion and protein misfolding science to Alberta to present lectures and to participate in panel discussions. These experts assist with planning or implementing collaborative research programs and/or explore and evaluate recent breakthroughs in prion science. Speakers might be involved in research related activities or public studies or both. Funds are provided primarily to offset the costs of travel and accommodation for guest speakers. Support of up to $1,500 is provided for speakers travelling within North America, and up to $2,500 is provided for speakers travelling from outside North America. In some cases, the Alberta Prion Research Institute will consider application for a contribution toward the cost of conducting an associated conference, symposia or workshop.
Application Deadline: continuous intake
EXPANDING HORIZONS PROGRAM
The Expanding Horizons Program is a professional development program for graduate students and postdoctoral fellows who are currently working on prion research. The objective of this program is to provide research trainees with access to mentoring in order to develop skills and knowledge that are applicable both within and outside of the lab. Seminars focus on career development in research as well as other careers they might undertake such as those in industry or policy making. Ensuring trainees are skilled in topics like lab management, networking, communicating, interview skills and grant crafting will benefit their futures as well as the future of prion research.
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic,
Tuesday, July 26, 2016
Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016
http://bse-atypical.blogspot.com/2016/07/atypical-bovine-spongiform.html
Tuesday, August 9, 2016
*** Concurrence with OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2015-0055]
http://bovineprp.blogspot.com/2016/08/concurrence-with-oie-risk-designations.html
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
http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
http://scrapie-usa.blogspot.com/2016/04/scrapie-ws-01-prion-diseases-in-animals.html
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.
http://jama.jamanetwork.com/article.aspx?articleid=1031186
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/content/60/2/176/reply#neurology_el_535
Sent: Monday, January 08,2001 3:03 PM
TO: freas@CBS5055530.CBER.FDA.GOV
FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January 2001 Meeting Singeltary Submission
2001 FDA CJD TSE Prion Singeltary Submission
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us ;;
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic,
what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
http://www.plosone.org/annotation/listThread.action?root=82860
Sunday, November 22, 2015
*** Effect of heating on the stability of amyloid A (AA) fibrils and the intra- and cross-species transmission of AA amyloidosis Abstract
Amyloid A (AA) amyloidosis is a protein misfolding disease characterized by extracellular deposition of AA fibrils. AA fibrils are found in several tissues from food animals with AA amyloidosis. For hygienic purposes, heating is widely used to inactivate microbes in food, but it is uncertain whether heating is sufficient to inactivate AA fibrils and prevent intra- or cross-species transmission. We examined the effect of heating (at 60 °C or 100 °C) and autoclaving (at 121 °C or 135 °C) on murine and bovine AA fibrils using Western blot analysis, transmission electron microscopy (TEM), and mouse model transmission experiments. TEM revealed that a mixture of AA fibrils and amorphous aggregates appeared after heating at 100 °C, whereas autoclaving at 135 °C produced large amorphous aggregates. AA fibrils retained antigen specificity in Western blot analysis when heated at 100 °C or autoclaved at 121 °C, but not when autoclaved at 135 °C. Transmissible pathogenicity of murine and bovine AA fibrils subjected to heating (at 60 °C or 100 °C) was significantly stimulated and resulted in amyloid deposition in mice. Autoclaving of murine AA fibrils at 121 °C or 135 °C significantly decreased amyloid deposition. Moreover, amyloid deposition in mice injected with murine AA fibrils was more severe than that in mice injected with bovine AA fibrils. Bovine AA fibrils autoclaved at 121 °C or 135 °C did not induce amyloid deposition in mice. These results suggest that AA fibrils are relatively heat stable and that similar to prions, autoclaving at 135 °C is required to destroy the pathogenicity of AA fibrils. These findings may contribute to the prevention of AA fibril transmission through food materials to different animals and especially to humans.
Purchase options Price * Issue Purchase USD 511.00 Article Purchase USD 54.00
http://www.tandfonline.com/doi/abs/10.3109/13506129.2015.1095735?journalCode=iamy20
http://betaamyloidcjd.blogspot.com/2015/11/effect-of-heating-on-stability-of.html
Posted by flounder on 05 Nov 2014 at 21:27 GMT
http://www.plosone.org/annotation/listThread.action?root=82860
Sunday, November 22, 2015
*** Effect of heating on the stability of amyloid A (AA) fibrils and the intra- and cross-species transmission of AA amyloidosis Abstract
Amyloid A (AA) amyloidosis is a protein misfolding disease characterized by extracellular deposition of AA fibrils. AA fibrils are found in several tissues from food animals with AA amyloidosis. For hygienic purposes, heating is widely used to inactivate microbes in food, but it is uncertain whether heating is sufficient to inactivate AA fibrils and prevent intra- or cross-species transmission. We examined the effect of heating (at 60 °C or 100 °C) and autoclaving (at 121 °C or 135 °C) on murine and bovine AA fibrils using Western blot analysis, transmission electron microscopy (TEM), and mouse model transmission experiments. TEM revealed that a mixture of AA fibrils and amorphous aggregates appeared after heating at 100 °C, whereas autoclaving at 135 °C produced large amorphous aggregates. AA fibrils retained antigen specificity in Western blot analysis when heated at 100 °C or autoclaved at 121 °C, but not when autoclaved at 135 °C. Transmissible pathogenicity of murine and bovine AA fibrils subjected to heating (at 60 °C or 100 °C) was significantly stimulated and resulted in amyloid deposition in mice. Autoclaving of murine AA fibrils at 121 °C or 135 °C significantly decreased amyloid deposition. Moreover, amyloid deposition in mice injected with murine AA fibrils was more severe than that in mice injected with bovine AA fibrils. Bovine AA fibrils autoclaved at 121 °C or 135 °C did not induce amyloid deposition in mice. These results suggest that AA fibrils are relatively heat stable and that similar to prions, autoclaving at 135 °C is required to destroy the pathogenicity of AA fibrils. These findings may contribute to the prevention of AA fibril transmission through food materials to different animals and especially to humans.
Purchase options Price * Issue Purchase USD 511.00 Article Purchase USD 54.00
http://www.tandfonline.com/doi/abs/10.3109/13506129.2015.1095735?journalCode=iamy20
http://betaamyloidcjd.blogspot.com/2015/11/effect-of-heating-on-stability-of.html
SWISS MEDICAL WEEKLY
Alzheimer-type brain pathology may be transmitted by grafts of dura mater 26/01/2016 Singeltary comment ;
http://blog.smw.ch/alzheimer-type-brain-pathology-may-be-transmitted-by-grafts-of-dura-mater/#comment-89016
re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
http://www.nature.com/nature/journal/v525/n7568/full/nature15369.html#/comments
Alzheimer-type brain pathology may be transmitted by grafts of dura mater 26/01/2016 Singeltary comment ;
http://blog.smw.ch/alzheimer-type-brain-pathology-may-be-transmitted-by-grafts-of-dura-mater/#comment-89016
re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
http://www.nature.com/nature/journal/v525/n7568/full/nature15369.html#/comments
Thursday, December 1, 2016
The European Union summary report on data of the surveillance of ruminants for the presence of transmissible spongiform encephalopathies (TSEs) in 2015
http://transmissiblespongiformencephalopathy.blogspot.com/2016/12/the-european-union-summary-report-on.html
Tuesday, November 29, 2016
Transmissibility of Gerstmann–Sträussler–Scheinker syndrome in rodent models: new insights into the molecular underpinnings of prion infectivity
http://transmissiblespongiformencephalopathy.blogspot.com/2016/11/transmissibility-of-gerstmannstraussler.html
Saturday, July 23, 2016
BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
http://bovineprp.blogspot.com/2016/07/bovine-spongiform-encephalopathy-bse.html
BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
http://bovineprp.blogspot.com/2016/07/bovine-spongiform-encephalopathy-bse.html
Tuesday, July 26, 2016
Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016
http://bse-atypical.blogspot.com/2016/07/atypical-bovine-spongiform.html
*** Calling Canadian beef unsafe is like calling your twin sister ugly," Dopp said.
Thursday, August 25, 2016
*** FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA MAD COW TYPE DISEASE ***
http://specifiedriskmaterial.blogspot.com/2016/08/fsis-green-bay-dressed-beef-recalls.html
Thursday, August 25, 2016
*** FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA MAD COW TYPE DISEASE ***
http://specifiedriskmaterial.blogspot.com/2016/08/fsis-green-bay-dressed-beef-recalls.html
Tuesday, August 9, 2016
*** Concurrence with OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2015-0055]
http://bovineprp.blogspot.com/2016/08/concurrence-with-oie-risk-designations.html
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
http://chronic-wasting-disease.blogspot.com/2016/05/zoonotic-potential-of-cwd-prions-update.html
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
http://chronic-wasting-disease.blogspot.com/2016/05/zoonotic-potential-of-cwd-prions-update.html
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
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
http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
http://scrapie-usa.blogspot.com/2016/04/scrapie-ws-01-prion-diseases-in-animals.html
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.
http://jama.jamanetwork.com/article.aspx?articleid=1031186
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/content/60/2/176/reply#neurology_el_535
Sent: Monday, January 08,2001 3:03 PM
TO: freas@CBS5055530.CBER.FDA.GOV
FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January 2001 Meeting Singeltary Submission
2001 FDA CJD TSE Prion Singeltary Submission
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us ;;
Terry S. Singeltary Sr.
Bacliff, Texas USA 77518
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