Review Article Prion Protein Misfolding, Strains, and Neurotoxicity: An Update from Studies on Mammalian Prions

Copyright © 2013 Ilaria Poggiolini et al.This is an open access article distributed under theCreative CommonsAttribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative disorders affecting humans and other mammalian species. The central event in TSE pathogenesis is the conformational conversion of the cellular prion protein, PrPC, into the aggregate