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Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins

Prions are unconventional infectious agents that are composed of misfolded aggregated prion protein. Prions replicate their conformation by template-assisted conversion of the endogenous prion protein PrP. Templated conversion of soluble proteins into protein aggregates is also a hallmark of other n...

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Detalles Bibliográficos
Autores principales: Krauss, Sybille, Vorberg, Ina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3825132/
https://www.ncbi.nlm.nih.gov/pubmed/24282413
http://dx.doi.org/10.1155/2013/704546
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author Krauss, Sybille
Vorberg, Ina
author_facet Krauss, Sybille
Vorberg, Ina
author_sort Krauss, Sybille
collection PubMed
description Prions are unconventional infectious agents that are composed of misfolded aggregated prion protein. Prions replicate their conformation by template-assisted conversion of the endogenous prion protein PrP. Templated conversion of soluble proteins into protein aggregates is also a hallmark of other neurodegenerative diseases. Alzheimer's disease or Parkinson's disease are not considered infectious diseases, although aggregate pathology appears to progress in a stereotypical fashion reminiscent of the spreading behavior ofmammalian prions. While basic principles of prion formation have been studied extensively, it is still unclear what exactly drives PrP molecules into an infectious, self-templating conformation. In this review, we discuss crucial steps in the life cycle of prions that have been revealed in ex vivo models. Importantly, the persistent propagation of prions in mitotically active cells argues that cellular processes are in place that not only allow recruitment of cellular PrP into growing prion aggregates but also enable the multiplication of infectious seeds that are transmitted to daughter cells. Comparison of prions with other protein aggregates demonstrates that not all the characteristics of prions are equally shared by prion-like aggregates. Future experiments may reveal to which extent aggregation-prone proteins associated with other neurodegenerative diseases can copy the replication strategies of prions.
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spelling pubmed-38251322013-11-26 Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins Krauss, Sybille Vorberg, Ina Int J Cell Biol Review Article Prions are unconventional infectious agents that are composed of misfolded aggregated prion protein. Prions replicate their conformation by template-assisted conversion of the endogenous prion protein PrP. Templated conversion of soluble proteins into protein aggregates is also a hallmark of other neurodegenerative diseases. Alzheimer's disease or Parkinson's disease are not considered infectious diseases, although aggregate pathology appears to progress in a stereotypical fashion reminiscent of the spreading behavior ofmammalian prions. While basic principles of prion formation have been studied extensively, it is still unclear what exactly drives PrP molecules into an infectious, self-templating conformation. In this review, we discuss crucial steps in the life cycle of prions that have been revealed in ex vivo models. Importantly, the persistent propagation of prions in mitotically active cells argues that cellular processes are in place that not only allow recruitment of cellular PrP into growing prion aggregates but also enable the multiplication of infectious seeds that are transmitted to daughter cells. Comparison of prions with other protein aggregates demonstrates that not all the characteristics of prions are equally shared by prion-like aggregates. Future experiments may reveal to which extent aggregation-prone proteins associated with other neurodegenerative diseases can copy the replication strategies of prions. Hindawi Publishing Corporation 2013 2013-10-26 /pmc/articles/PMC3825132/ /pubmed/24282413 http://dx.doi.org/10.1155/2013/704546 Text en Copyright © 2013 S. Krauss and I. Vorberg. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Krauss, Sybille
Vorberg, Ina
Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title_full Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title_fullStr Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title_full_unstemmed Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title_short Prions Ex Vivo: What Cell Culture Models Tell Us about Infectious Proteins
title_sort prions ex vivo: what cell culture models tell us about infectious proteins
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3825132/
https://www.ncbi.nlm.nih.gov/pubmed/24282413
http://dx.doi.org/10.1155/2013/704546
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