CAS NE 525 Lecture Notes - Lecture 12: Scrapie, Jugular Vein, Glycosylphosphatidylinositol

• School: Boston University
• Department: Neuroscience
• Course: CAS NE 525
• Professor: Lucia Pastorino

Document Summary

Transmissible spongiform encephalopathy (tse) infectious agent is prion (prpc converted to prpsc) Physiologic prpc (cellular form) to pathologic prpsc (scrappy form) gain of. Scrappy form can cause cellular form to change into toxic scrappy form very resistant to heat, radiation, disinfection, and protein digestion (degradation) Spongiform (intracellular vacuolation) leads to vacuole formation in grey matter near deposition of prion protein brain shrinkage and deterioration massive neurodegeneration. Model for conformational conversion of prpc into prpsc. Scrappy form is same protein as cellular protein from amino acid point of view but something went wrong in folding association with mutations or random events that triggered misfolding. Once misfolded into scrappy form (beta sheet conformation), they have the ability to induce misfolding of cellular forms (transmissible) infectious agent (prpsc) has ability to induce conformational changes on physiologically folded protein. It could take decades before carrier becomes fully symptomatic. Because prpsc is resistant to protein digestion allows for early detection before symptoms show.