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The biological function of the cellular prion protein: an update
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 function...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5412054/ https://www.ncbi.nlm.nih.gov/pubmed/28464931 http://dx.doi.org/10.1186/s12915-017-0375-5 |
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author | Wulf, Marie-Angela Senatore, Assunta Aguzzi, Adriano |
author_facet | Wulf, Marie-Angela Senatore, Assunta Aguzzi, Adriano |
author_sort | Wulf, Marie-Angela |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5412054 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-54120542017-05-03 The biological function of the cellular prion protein: an update Wulf, Marie-Angela Senatore, Assunta Aguzzi, Adriano BMC Biol Review 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. BioMed Central 2017-05-02 /pmc/articles/PMC5412054/ /pubmed/28464931 http://dx.doi.org/10.1186/s12915-017-0375-5 Text en © Aguzzi et al. 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Review Wulf, Marie-Angela Senatore, Assunta Aguzzi, Adriano The biological function of the cellular prion protein: an update |
title | The biological function of the cellular prion protein: an update |
title_full | The biological function of the cellular prion protein: an update |
title_fullStr | The biological function of the cellular prion protein: an update |
title_full_unstemmed | The biological function of the cellular prion protein: an update |
title_short | The biological function of the cellular prion protein: an update |
title_sort | biological function of the cellular prion protein: an update |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5412054/ https://www.ncbi.nlm.nih.gov/pubmed/28464931 http://dx.doi.org/10.1186/s12915-017-0375-5 |
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