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The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls

Prion diseases are a family of transmissible, progressive, and uniformly fatal neurodegenerative disorders that affect humans and animals. Although cross-species transmissions of prions are usually limited by an apparent “species barrier”, the spread of a prion disease to humans by ingestion of cont...

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Detalles Bibliográficos
Autores principales: Acevedo-Morantes, Claudia Y., Wille, Holger
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213568/
https://www.ncbi.nlm.nih.gov/pubmed/25333467
http://dx.doi.org/10.3390/v6103875
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author Acevedo-Morantes, Claudia Y.
Wille, Holger
author_facet Acevedo-Morantes, Claudia Y.
Wille, Holger
author_sort Acevedo-Morantes, Claudia Y.
collection PubMed
description Prion diseases are a family of transmissible, progressive, and uniformly fatal neurodegenerative disorders that affect humans and animals. Although cross-species transmissions of prions are usually limited by an apparent “species barrier”, the spread of a prion disease to humans by ingestion of contaminated food, or via other routes of exposure, indicates that animal prions can pose a significant public health risk. The infectious agent responsible for the transmission of prion diseases is a misfolded conformer of the prion protein, PrP(Sc), a pathogenic isoform of the host-encoded, cellular prion protein, PrP(C). The detailed mechanisms of prion conversion and replication, as well as the high-resolution structure of PrP(Sc), are unknown. This review will discuss the general background related to prion biology and assess the structural models proposed to date, while highlighting the experimental challenges of elucidating the structure of PrP(Sc).
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spelling pubmed-42135682014-10-31 The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls Acevedo-Morantes, Claudia Y. Wille, Holger Viruses Review Prion diseases are a family of transmissible, progressive, and uniformly fatal neurodegenerative disorders that affect humans and animals. Although cross-species transmissions of prions are usually limited by an apparent “species barrier”, the spread of a prion disease to humans by ingestion of contaminated food, or via other routes of exposure, indicates that animal prions can pose a significant public health risk. The infectious agent responsible for the transmission of prion diseases is a misfolded conformer of the prion protein, PrP(Sc), a pathogenic isoform of the host-encoded, cellular prion protein, PrP(C). The detailed mechanisms of prion conversion and replication, as well as the high-resolution structure of PrP(Sc), are unknown. This review will discuss the general background related to prion biology and assess the structural models proposed to date, while highlighting the experimental challenges of elucidating the structure of PrP(Sc). MDPI 2014-10-20 /pmc/articles/PMC4213568/ /pubmed/25333467 http://dx.doi.org/10.3390/v6103875 Text en © 2014 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Acevedo-Morantes, Claudia Y.
Wille, Holger
The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title_full The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title_fullStr The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title_full_unstemmed The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title_short The Structure of Human Prions: From Biology to Structural Models — Considerations and Pitfalls
title_sort structure of human prions: from biology to structural models — considerations and pitfalls
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213568/
https://www.ncbi.nlm.nih.gov/pubmed/25333467
http://dx.doi.org/10.3390/v6103875
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