Sequestration of DROSHA and DGCR8 by Expanded CGG RNA Repeats Alters MicroRNA Processing in Fragile X-Associated Tremor/Ataxia Syndrome期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

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

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

Affiliation:	1. Department of Translational Medicine, IGBMC, Illkirch 67400, France;2. College de France, Paris 75000, France;3. Department of Chemistry, The Scripps Research Institute, Jupiter, FL 33458, USA;4. Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA;5. M.I.N.D. Institute, University of California, Davis, Sacramento, CA 95817, USA;6. Department of Biochemistry and Molecular Medicine, University of California, Davis, Sacramento, CA 95817, USA;7. Department of Clinical Genetics, Erasmus MC, Rotterdam 3000 DR, The Netherlands

Abstract:	Download : Download full-size image Highlights? DGCR8 binds to CGG RNA repeats, cause of the neurodegenerative FXTAS disease ? DGCR8 and its partner, DROSHA, are sequestered within CGG RNA aggregates ? DGCR8 rescues the neuronal cell death induced by expanded CGG RNA repeats ? MicroRNA processing is impaired in patients with FXTAS

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