The role of lipid rafts in prion protein biology

Abstract

The conformational conversion of the cellular prion protein, PrP C, to the misfolded isoform PrPSc is the central pathogenic event in the uniquely transmissible neurodegenerative prion diseases. As both PrPC and PrPSc are associated with membranes, the nature of the membrane microenvironment may well play a significant role in both the conformational conversion process as well as the normal functions of PrPC. Within the membrane are various microdomains, areas of distinct lipid and protein composition, the best studied of which are the cholesteroland sphingolipid-rich lipid rafts. These domains are characterized biochemically by their relative resistance to solubilization in certain detergen..