Year: 2017
Type: review
Abstract: 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.
Source: BMC Biology
Institution: University of Zurich
Language: English
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $3,690
OpenAlex ID: W2611707378