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Mutations Alter RNA-Mediated Conversion of Human Prions

[Image: see text] Prion diseases are connected with self-replication and self-propagation of misfolded proteins. The rate-limiting factor is the formation of the initial seed. We have recently studied the early stages in the conversion between functional PrP(C) and the infectious scrapie PrP(SC) for...

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Autores principales: Alred, Erik J., Lodangco, Izra, Gallaher, Jennifer, Hansmann, Ulrich H.E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Chemical Society 2018
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5928492/
https://www.ncbi.nlm.nih.gov/pubmed/29732450
http://dx.doi.org/10.1021/acsomega.7b02007
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author Alred, Erik J.
Lodangco, Izra
Gallaher, Jennifer
Hansmann, Ulrich H.E.
author_facet Alred, Erik J.
Lodangco, Izra
Gallaher, Jennifer
Hansmann, Ulrich H.E.
author_sort Alred, Erik J.
collection PubMed
description [Image: see text] Prion diseases are connected with self-replication and self-propagation of misfolded proteins. The rate-limiting factor is the formation of the initial seed. We have recently studied the early stages in the conversion between functional PrP(C) and the infectious scrapie PrP(SC) form, triggered by the binding of RNA. Here, we study how this process is modulated by the prion sequence. We focus on residues 129 and 178, which are connected to the hereditary neurodegenerative disease fatal familial insomnia.
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spelling pubmed-59284922018-05-02 Mutations Alter RNA-Mediated Conversion of Human Prions Alred, Erik J. Lodangco, Izra Gallaher, Jennifer Hansmann, Ulrich H.E. ACS Omega [Image: see text] Prion diseases are connected with self-replication and self-propagation of misfolded proteins. The rate-limiting factor is the formation of the initial seed. We have recently studied the early stages in the conversion between functional PrP(C) and the infectious scrapie PrP(SC) form, triggered by the binding of RNA. Here, we study how this process is modulated by the prion sequence. We focus on residues 129 and 178, which are connected to the hereditary neurodegenerative disease fatal familial insomnia. American Chemical Society 2018-04-09 /pmc/articles/PMC5928492/ /pubmed/29732450 http://dx.doi.org/10.1021/acsomega.7b02007 Text en Copyright © 2018 American Chemical Society This is an open access article published under an ACS AuthorChoice License (http://pubs.acs.org/page/policy/authorchoice_termsofuse.html) , which permits copying and redistribution of the article or any adaptations for non-commercial purposes.
spellingShingle Alred, Erik J.
Lodangco, Izra
Gallaher, Jennifer
Hansmann, Ulrich H.E.
Mutations Alter RNA-Mediated Conversion of Human Prions
title Mutations Alter RNA-Mediated Conversion of Human Prions
title_full Mutations Alter RNA-Mediated Conversion of Human Prions
title_fullStr Mutations Alter RNA-Mediated Conversion of Human Prions
title_full_unstemmed Mutations Alter RNA-Mediated Conversion of Human Prions
title_short Mutations Alter RNA-Mediated Conversion of Human Prions
title_sort mutations alter rna-mediated conversion of human prions
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5928492/
https://www.ncbi.nlm.nih.gov/pubmed/29732450
http://dx.doi.org/10.1021/acsomega.7b02007
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