The biological function of the cellular prion protein: an update

• Main Authors: Marie-Angela Wulf, Assunta Senatore, Adriano Aguzzi
• Format: Article
• Language: English
• Published: BMC 2017-05-01
• Series: BMC Biology
• Subjects: Prion Disease; Metabotropic Glutamate Receptor; Cellular Prion Protein; Prnp Gene; Flexible Tail
• Online Access: http://link.springer.com/article/10.1186/s12915-017-0375-5

Abstract The misfolding of the cellular prion protein (PrPC) causes fatal neurodegenerative diseases. Yet PrPC is highly conserved in mammals, suggesting that it exerts beneficial functions preventing its evolutionary elimination. Ablation of PrPC in mice results in well-defined structural and functional alterations in the peripheral nervous system. Many additional phenotypes were ascribed to the lack of PrPC, but some of these were found to arise from genetic artifacts of the underlying mouse models. Here, we revisit the proposed physiological roles of PrPC in the central and peripheral nervous systems and highlight the need for their critical reassessment using new, rigorously controlled animal models.