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C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression

Expansion of an intronic (GGGGCC)(n) repeat region within the C9orf72 gene is a main cause of familial amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD). A hallmark of c9ALS/FTD is the accumulation of misprocessed RNAs, which are often targets of cellular RNA surveillance. Here,...

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Detalles Bibliográficos
Autores principales: Sun, Yu, Eshov, Aziz, Zhou, Jeffrey, Isiktas, Atagun U., Guo, Junjie U.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335171/
https://www.ncbi.nlm.nih.gov/pubmed/32620797
http://dx.doi.org/10.1038/s41467-020-17129-0