Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent ISR in C9ORF72 FTD/ALS

Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent ISR in C9ORF72 FTD/ALS

GGGGCC (G4C2) hexanucleotide repeat expansion in the C9ORF72 gene is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The repeat is bidirectionally transcribed and confers gain of toxicity. However, the underlying toxic species is debated, and i...

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Bibliographic Details
Main Authors: Janani Parameswaran, Nancy Zhang, Elke Braems, Kedamawit Tilahun, Devesh C Pant, Keena Yin, Seneshaw Asress, Kara Heeren, Anwesha Banerjee, Emma Davis, Samantha L Schwartz, Graeme L Conn, Gary J Bassell, Ludo Van Den Bosch, Jie Jiang
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2023-04-01
Series:eLife
Subjects:
C9ORF72
PKR
antisense RNA
FTD/ALS
integrated stress response
Online Access:https://elifesciences.org/articles/85902

Internet

https://elifesciences.org/articles/85902

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