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

GGGGCC (G(4)C(2)) 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, a...

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Detalles Bibliográficos
Autores principales: Parameswaran, Janani, Zhang, Nancy, Braems, Elke, Tilahun, Kedamawit, Pant, Devesh C, Yin, Keena, Asress, Seneshaw, Heeren, Kara, Banerjee, Anwesha, Davis, Emma, Schwartz, Samantha L, Conn, Graeme L, Bassell, Gary J, Van Den Bosch, Ludo, Jiang, Jie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2023
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10188109/
https://www.ncbi.nlm.nih.gov/pubmed/37073950
http://dx.doi.org/10.7554/eLife.85902

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