Prion Protein and the Family Members, Doppel and Shadoo
from: Prions: Current Progress in Advanced Research (Edited by: Akikazu Sakudo and Takashi Onodera). Caister Academic Press, U.K. (2013)
Prion diseases are devastating neurodegenerative disorders caused by infectious proteinaceous agents known as prions. Prion protein (PrP) gene (Prnp)-deficient mice do not infect with prion agent, indicating essential role of PrP for prion diseases. An abnormal isoform of prion protein (PrP), known as PrPSc, which is converted from cellular PrP (PrPC), is thought to constitute the prion agent. Recently, proteins homologous to PrP have been found, suggesting the existence of other PrP family members, which so far include PrP, Doppel (Dpl) and Shadoo (Sho). In this chapter, the author introduces recent research on the physiological function of PrP and PrP-related proteins together with our own studies read more ...