ABSTRACT
The evolutionary conservation of the prion protein (PrP) primary structure among mammals, its high level of expression in brain tissue, and its localization in highly specialized membrane domains suggest that the normal cellular protein (PrPC) may play an important biological function. It is not yet known whether changes in the putative biological activity of PrPC are implicated in the pathogenesis [Hetz et al., 2003]. This chapter describes the cell biology, biosynthesis, and cellular localization of PrPC as well as the several putative biological activities associated with it. The contribution of animal models to our understanding of PrPC biological activity and its participation in TSE (transmissible spongiform encephalopathy) pathogenesis is addressed at the end of the chapter.
