Microbiology and Immunology 2500A/B Lecture Notes - Lecture 1: Bovine Spongiform Encephalopathy, Amyloid, Scrapie
5 Aug 2018
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Document Summary
The prion theory the protein only hypothesis for scrapie in a stand-alone paper by stanley prisoner. Tses were initially thought to be caused by an unidentified slow virus. Prion: form of a host protein found predominantly on the surface of neurons. The cellular protein is called prpc: normal functions to well understood; maybe synaptic function, not infectious. Infectious protein called prpsc (modified version of prpc) the smallest known infectious agent: proteinaceous infectious particles: contain no nucleic acid, no immune response provoked, does not self-replicate (needs to convert host in non- reversible conformational change) Accumulation of prions in the brain causes neurons to die. Formation of amyloid plaques; insoluble fibrous protein aggregates. The destruction of neural cells causes tiny holes in brain tissues ( spongiform disease) Symptoms: anything involving brain function (moment, personality, impairments, etc. ) Transmission is likely from ewes to offspring but is not well understood (not to humans) A major problem in the uk until mid-1990s.
