Trafficking of the Cellular Prion Protein and Its Role in Neurodegeneration

摘要: Publisher Summary Some of the most debilitating neurodegenerative diseases are result a seemingly unassuming protein that has deviated from normal pathways biosynthesis, trafficking, and degradation characterize its lifetime. The ensuing consequences manifest over remarkably long periods time as complex molecular cascades conclude with cellular dysfunction death. This chapter examines this concept for particularly enigmatic set caused by prion (PrP). Although basis these is far clear, illustrates, using available information, how key to interpreting lies in understanding protein-trafficking pathways. PrP biosynthesis metabolism constitute complex, variable, regulated events generate wide range species proteome. Alterations amounts, subcellular location, or temporal expression certain members proteome likely play critical role pathogenesis PrP-associated diseases. A valuable immediate aim study thoroughly define proteome, delineate which individual components within it generated, develop methods manipulate track notable species.