PHYS 799 Lecture Notes - Lecture 59: Fatal Familial Insomnia, Chronic Wasting Disease, Paraplegia
12 Jun 2019
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Document Summary
Prion = infectious protein that induces self-propagating change in conformation. Normal form = prpc, abnormally folded form = prpsc. Prpc mostly alpha helix, protease sensitive, in plasma membrane; prpsc mostly beta sheet, protease resistant, in lysosomes. Prpsc can form spontaneously and catalyze the rapid conversion of prpc to prpsc, leading to amyloid formation. Amyloid = protein aggregates that accumulate as fibers, stain w/congo red. Spongiform encephalopathies are prion diseases can be transmitted, sporadic, or inherited. Spongiform = vacuoles are formed in the brain tissue. Examples: kuru (new guinea cannibals oral transmission), creutzfeld-jakob disease (cjd transmitted, sporadic, inherited; progressive dementia), gerstmann-straussler-scheinker syndrome (gss inherited, sporadic; ataxia, paraparesis, dementia), fatal familial insomnia (ffi. Inherited, sporadic), animal spongiform encephalopathies (scrapie in sheep, chronic wasting disease in deer/elk, bovine spongiform encephalopathy in cows) of the animal ones, only bse can be transmitted to humans ( variant cjd) Slightly different prions (but all are still prpsc) involved different histology and localization.
