UC 154 Lecture Notes - Lecture 7: Bovine Spongiform Encephalopathy, Fatal Familial Insomnia, Chronic Wasting Disease
Document Summary
Prions are, to say the least, unconventional infectious agents. Today we"ll talk about what they are, how they replicate, how they spread, and their link to both infectious and non-infectious disease. What does the name prion stand for? proteinaceous infectious particle. What are the physical properties of prions? mis-folded prion proteins highly structured self propagating (breeding/cultivating) Where do prions come from? derive from the self-propagating modification of endogenous proteins. However, they can also occur through protein mis-folding and autocatalytic assembly of proteins. Caused by eating human brain tissue contaminated with infectious prions. Because of increased awareness about the disease it is now rare. Variant cjd - reated to mad cow disease . Variably protease- sensitive prionopathy (vpspr) - rare but similar to cjd. How do prions reach the organ where they cause disease? hereditary infection from contaminated medical tools - prions can resist normal sterilization.
