The biological function of the cellular prion protein: an update

作者：Marie-Angela Wulf, Assunta Senatore, Adriano Aguzzi · 发表于：BMC Biology · 年份：2017 · DOI：10.1186/s12915-017-0375-5 · 被引用次数：270 · 研究领域：Prion Diseases and Protein Misfolding、Neurological diseases and metabolism、Trace Elements in Health

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