HMB360H1 Study Guide - Final Guide: Bovine Spongiform Encephalopathy, Multiple System Atrophy, Transmissible Spongiform Encephalopathy
Document Summary
Lecture 6 cellular & molecular biology of prions. Dr. carleton went to papua new guinea, met people who had trembling in members of a tribe had kuru, transmitted via cannibalism. We(cid:374)t to (cid:448)et a(cid:374)d he said so(cid:373)e sheep ha(cid:448)e sa(cid:373)e shaki(cid:374)g; (cid:272)alled (cid:862)scrapie(cid:863) i(cid:374) sheep, the(cid:455) rub against things and scrape fur off. Looked at their brains of sheep, looked like a sponge spongiform brain. Prions are not viruses, no genetic material passed from one brain to another. Prion = proteinaceous infectious particle, only made of protein. Resistant to nucleases, wt not resistant to proteases. Common form of all prion disorders cause transmissible spongiform encephalopathy (tses) Creutzfeldt-jakob disease (cjd) + scrapie (sheep) + kuru (human) misfolded protein prpsc (the scrapie form of the prion protein) | prpc (cellular prion protein normal) Did not know how a prion hypothesis made sense, since the disorders were infectious, did not understand how a protein could replicate without viral nucleic acid genome.
