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Reversibility of neuropathology and motor deficits in an inducible mouse model for FXTAS

Archive ouverte | Hukema, Renate | CCSD

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Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome

Archive ouverte | Berman, Robert F | CCSD

International audience. Carriers of the fragile X premutation (FPM) have CGG trinucleotide repeat expansions of between 55 and 200 in the 5′-UTR of FMR1, compared to a CGG repeat length of between 5 and 54 for the g...

Translation of Expanded CGG Repeats into FMRpolyG Is Pathogenic and May Contribute to Fragile X Tremor Ataxia Syndrome

Archive ouverte | Sellier, Chantal | CCSD

International audience. Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by a limited expansion of CGG repeats in the 5' UTR of FMR1. Two mechanisms are proposed to cause FX...

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