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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Detalles Bibliográficos
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
Descripción
Sumario:[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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