Biochemistry 3381A Study Guide - Quiz Guide: Transmissible Spongiform Encephalopathy, Stanley B. Prusiner, Amyloid
20 May 2020
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Induce transmissible spongiform encephalopathies (tses: gross structure: vacuoles in brain. Epidemics" => infectious agent: alders + gajdusek infected chimp w/ brain material from human. Kuru victim: creutzfeldt-jakob disease (cjd) + variant/ new cjd man. Couldn"t find bacterium / virus: survived irradiation destroy nucleic acid. Stanley prusiner prion hypothesis 1997 nobel: identified protein = prp (prion protein) 1976 gadjusek > nobel kuru transmissible e. g. to chimps. The seeding prion generates more of the same misfolded: insert bse prion into mouse > mouse bse misfolding. Prpsc scrapie: mainly beta sheet, stable covalent linkages protease k resistant, accumulates: promote normal misfolding, neural accumulation > amyloid plaques. Eating infected e. g. kuru cannibalism (worsened by specific consumption of cns- [highest]: bse cattle animal fed to animal, vcjd eat infected meat. Iatrogenic: medical procedure instrument contamination or via blood: v hard to fully remove protein (single molecule can seed) > incinerate possible contaminant. Cattle fed meat and bone meal (mbm) prions scrapie sheep.
