Cell Biology Approaches to Studying Prion Diseases.

During the course of prion infection, the normally soluble and protease-sensitive mammalian prion protein (PrP ^C ) is refolded into an insoluble, partially protease-resistant, and infectious form called PrP ^Sc . The conformational conversion of PrP ^C to PrP ^Sc is a critical event during prion infection and is essential for the production of prion infectivity. This chapter briefly summarizes the ways in which cell biological approaches have enhanced our understanding of how PrP contributes to different aspects of prion pathogenesis.