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

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, th...

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Main Authors: Chantal Sellier, Fernande Freyermuth, Ricardos Tabet, Tuan Tran, Fang He, Frank Ruffenach, Violaine Alunni, Herve Moine, Christelle Thibault, Adeline Page, Flora Tassone, Rob Willemsen, Matthew D. Disney, Paul J. Hagerman, Peter K. Todd, Nicolas Charlet-Berguerand
Format: Article
Language:English
Published: Elsevier 2013-03-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124713000636

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