Cargando…
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...
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: | |
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 |
Ejemplares similares
-
C9orf72 loss-of-function: a trivial, stand-alone or additive mechanism in C9 ALS/FTD?
por: Braems, Elke, et al.
Publicado: (2020) -
Metformin inhibits RAN translation through PKR pathway and mitigates disease in C9orf72 ALS/FTD mice
por: Zu, Tao, et al.
Publicado: (2020) -
Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD
por: Moens, Thomas G., et al.
Publicado: (2018) -
Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit
por: Sattler, Rita, et al.
Publicado: (2023) -
The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD
por: Freibaum, Brian D., et al.
Publicado: (2017)