Mutations Alter RNA-Mediated Conversion of Human Prions | Zendy

Erik J. Alred | Zendy; Izra D. Lodangco | Zendy; Jennifer Gallaher | Zendy; Ulrich H. E. Hansmann | Zendy

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Mutations Alter RNA-Mediated Conversion of Human Prions

Author(s) -

Erik J. Alred,

Izra D. Lodangco,

Jennifer Gallaher,

Ulrich H. E. Hansmann

Publication year - 2018

Publication title -

acs omega

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.779

H-Index - 40

ISSN - 2470-1343

DOI - 10.1021/acsomega.7b02007

Subject(s) - scrapie , rna , fatal familial insomnia , limiting , prion protein , mutation , biology , virology , rna processing , sequence (biology) , microbiology and biotechnology , genetics , gene , disease , medicine , mechanical engineering , pathology , engineering

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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