Glenn Telling analyzes Prion Diseases through Transgenic Mouse Modeling and discusses his studies during the CJD (Creutzfeldt-Jakob Disease) Foundation Annual Family Conference, 2015.
View conference videos here: [ Ссылка ]
0:49 - Long Term Goals of Prion Diseases
1:47 - Working Hypothesis
3:30 - Experimental Transmission of Prion Diseases
4:35 - Experimental Transmission of Prions to Mice
4:45 - Prion Species Barrier
5:30 - Identification of Prion Strains
7:24 - Transgenic Mouse Modeling
8:55 - Inherited Human Prion Diseases
11:34 - Chronic Wasting Disease (CWD)
13:20 - Addressing CWD
17:38 - Conclusions
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Watch more from this conference at:
2015 CJD Foundation Family Conference Playlist: [ Ссылка ]
Individual Presentations:
Byron Caughey - Detecting Prions: [ Ссылка ]
Glenn Telling - Transgenic Mouse Modeling: [ Ссылка ]
Thomas Wisniewski - Vaccination Approaches: [ Ссылка ]
Q&A with Caughey, Telling, Wisniewski, Will: [ Ссылка ]
Jean-Phillipe Deslys: Human Mini-Brains: [ Ссылка ]
Julia Moreno: Gerstamann-Straussler Scheinker Syndrome: [ Ссылка ]
Chrsitina Sigurdson - Selective Cell Vulnerability: [ Ссылка ]
Q&A with Deslys, Moreno, Sigurdson, and Knight: [ Ссылка ]
Emiliano Biasini - Pharmacological Chaperones [ Ссылка ]
CJD Foundation Website: [ Ссылка ]
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"Prion diseases are a group of rare, invariably fatal brain disorders which occur both in humans and certain animals. They first came to public attention in the mid 1980s in the form of the BSE epidemic in the United Kingdom. BSE (bovine spongiform encephalopathy) is a prion disease in cattle. Tissue from infected animals may have contaminated cattle feed, leading to the silent spread of the BSE epidemic. There is also a theory that BSE came from feed contaminated with scrapie, the long established sheep prion disease. Inevitably, concern over whether BSE could pass to humans mounted.
In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one person per million per year. In the United States this translates to 250-300 new cases per year. It is well known that CJD is very difficult to diagnose leading to speculation that the one case per million report may be incorrect. Most of the cases are "classical" or "sporadic" CJD (sCJD), occurring for no, as yet, known reason. The sporadic form accounts for approximately 85% of the cases, the familial form approximately 15%. There have also been a few cases which have occurred from contamination via medical procedures; this type is known as iatrogenic or Acquired CJD. Finally over the last few years, another type of Acquired CJD called variant (vCJD) has been identified in young people. CJD has been linked to ingestion of beef tainted with BSE (bovine spongiform encephalopathy), most cases have occurred in the United Kingdom."
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