Prion protein and its role in signal transduction

Prion diseases are a class of fatal neurodegenerative disorders that can be sporadic, genetic or iatrogenic. They are characterized by the unique nature of their etiologic agent: prions (PrP(Sc)). A prion is an infectious protein with the ability to convert the host-encoded cellular prion protein (PrP(C)) into new prion molecules by acting as a template. Since Stanley B. Prusiner proposed the “protein-only” hypothesis for the first time, considerable effort has been put into defining the role played by PrP(C) in neurons. However, its physiological function remains unclear. This review summarizes the major findings that support the involvement of PrP(C) in signal transduction.