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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 1700’s Scrapie described in sheep 1920 CJD described in humans 1939 Experimental transmission of scrapie History of Prion Disease 1700’s 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 1960’s 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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  • Winter '09
  • Fortes
  • Prion, Transmissible spongiform encephalopathy, prion disease, PrP gene, Scrapie, Experimental transmission

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