BICH 410 Lecture Notes - Lecture 12: Bovine Spongiform Encephalopathy, Sickle-Cell Disease, Cystic Fibrosis
Document Summary
When proteins go through misfolding they can either aggregate or continually degrade Protein affected: cystic brosis transmembrane conductance regulator (cftr) Prion diseases - affects the nervous system in humans and animals. The disease cause deteriorating affects on the brain. Cows- mad cow disease (able to be transmitted to humans) Scrapies & mad cow disease are similar enough in structure that they are able to affect the other species. Found on surface of certain cells very soluble. Digested by proteases encoded by gene designated in human on chromosome 20. Forces prpc to form into a version of itself when in close proximity. Beta-amyloid peptide- derivative of a much large membrane bound protein called - amyloid precursor protein (app) Occurs by the interaction of partially unfolded proteins that then form extensive. Beta sheets that are virtually indestructible -> the formation of the amyloid bers leads to alzheimers The formations kill off healthy neurons within the brain.
