Sequestration of DROSHA and DGCR8 by Expanded CGG RNA Repeats Alters MicroRNA Processing in Fragile X-Associated Tremor/Ataxia Syndrome

Fragile X-associated tremor/ataxia syndrome (FXTAS) is an inherited neurodegenerative disorder caused by the expansion of 55–200 CGG repeats in the 5′ UTR of FMR1. These expanded CGG repeats are transcribed and accumulate in nuclear RNA aggregates that sequester one or more RNA-binding proteins, thu...

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Autori principali: Sellier, Chantal, Freyermuth, Fernande, Tabet, Ricardos, Tran, Tuan, He, Fang, Ruffenach, Frank, Alunni, Violaine, Moine, Herve, Thibault, Christelle, Page, Adeline, Tassone, Flora, Willemsen, Rob, Disney, Matthew D., Hagerman, Paul J., Todd, Peter K., Charlet-Berguerand, Nicolas
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2013
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3639429/
https://ncbi.nlm.nih.gov/pubmed/23478018
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.celrep.2013.02.004
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