BIPN150.L16.Prion

BIPN150.L16.Prion - Click to edit Master subtitle style...

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Unformatted text preview: Click to edit Master subtitle style Lecture 16: Prion Diseases : Spongiform Encephalopathies March 3, 2009 Prion = proteinaceous infective particle, neurodegnerative, slow (years), lack of immune response/inflammation, brain cells (neurons and glia) contain vacuoles (i.e. spongy). Human Prion Diseases Kuru Creutzfeld-Jakob disease (CJD): sporadic (85%), inherited (15%) Animal Prion Diseases-- Scrapie (sheep) Transmissible mink encephalopathy (TME) Bovine Spongiform encephalopathy (Mad cow) Background Background History of Prion Disease 1700s Scrapie described in sheep 1920 CJD described in humans 1939 Experimental transmission of scrapie History of Prion Disease 1700s Scrapie described in sheep 1920 CJD described in humans 1939 Experimental transmission of scrapie 1966/68 Kuru/CJD transmission to primates From: Collinge et al. , The Lancet vol. 367, pp. 2068-2074, 2006. Kuru Disease 1960s D. Carlton Gajdusek Nobel Prize award in 1976 Fore language: Kuru=shiver Chronic degeneration: ataxia dementia Infectious - but non-conventional slow virus no immune response no inflammation Kuru Creutzfeld Jacob Disease (1:1,000,000) Scrapie Spongiform Encephalitis (transmissible): TSE Spongiform Encephalitis Normal Click to edit Master subtitle style Stanley Prusiner was a doctor at UCSF in the 1970s and had a patient with CJD. Started to read about it and became interested in purifying the infectious agent related to CJD Used Scrapie (sheep) infection in hampsters as model Highly purified protein fractions resistant to UV light degraded by proteinases Coined the term Prion = proteinaceous infective agent PRION: Agent of transmissible spongiform encephalopathy (TSE), with unconventional properties....
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This note was uploaded on 04/01/2009 for the course BIPN 150 taught by Professor Fortes during the Winter '09 term at UCSD.

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BIPN150.L16.Prion - Click to edit Master subtitle style...

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