CGG repeat RNA G-quadruplexes interact with FMRpolyG to cause neuronal dysfunction in fragile X-related tremor/ataxia syndrome

Fragile X-related tremor/ataxia syndrome (FXTAS) is a neurodegenerative disease caused by CGG triplet repeat expansions in FMR1, which elicit repeat-associated non-AUG (RAN) translation and produce the toxic protein FMRpolyG. We show that FMRpolyG interacts with pathogenic CGG repeat-derived RNA G-q...

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Published in:Sci Adv
Main Authors: Asamitsu, Sefan, Yabuki, Yasushi, Ikenoshita, Susumu, Kawakubo, Kosuke, Kawasaki, Moe, Usuki, Shingo, Nakayama, Yuji, Adachi, Kaori, Kugoh, Hiroyuki, Ishii, Kazuhiro, Matsuura, Tohru, Nanba, Eiji, Sugiyama, Hiroshi, Fukunaga, Kohji, Shioda, Norifumi
Format: Artigo
Language:Inglês
Published: American Association for the Advancement of Science 2021
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Research Articles
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC7806243/
https://ncbi.nlm.nih.gov/pubmed/33523882
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/sciadv.abd9440
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