Mutations Alter RNA-Mediated Conversion of Human Prions.
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ABSTRACT:
SUBMITTER: Alred EJ
PROVIDER: S-EPMC5928492 | biostudies-literature | 2018 Apr
REPOSITORIES: biostudies-literature
Publications
Mutations Alter RNA-Mediated Conversion of Human Prions.
Alred Erik J EJ Lodangco Izra I Gallaher Jennifer J Hansmann Ulrich H E UHE
ACS omega 20180409 4
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<sup>C</sup> and the infectious scrapie PrP<sup>SC</sup> 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 diseas ...[more]
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