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Article

CGG-repeat length threshold for FMR1 RNA pathogenesis in a cellular model for FXTAS.

Human molecular genetics - 1 Jun 2011

Hoem Gry, Raske Christopher R, Garcia-Arocena Dolores, Tassone Flora, Sanchez Eleonora, Ludwig Anna L, Iwahashi Christine K, Kumar Madhur, Yang Jane E, Hagerman Paul J

Abstract excerpt

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder that affects carriers of premutation alleles (55-200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene. The presence of elevated levels of expanded mRNA found in premutation carriers is believed to be the basis for the pathogenesis in FXTAS, but the exact mechanisms by which the mRNA causes toxicity are not known. In...

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Identifiers

PMID: 21389081
DOI: 10.1093/hmg/ddr101
PMCID: PMC3090194

Genes

FMR1

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