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:
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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