RNA-mediated neurodegeneration in fragile X-associated tremor/ataxia syndrome.

Abstract excerpt

Carriers of fragile X syndrome (FXS) have FMR1 alleles, called premutations, with a number of 5'-untranslated-CGG repeats somewhere between patients, who have over 200 repeats, and normal individuals, with fewer than 60 repeats. Fragile X-associated tremor/ataxia syndrome (FXTAS), a late-onset neurodegenerative disorder, has been recognized in older male fragile X premutation carriers, and FXTAS is uncoupled from...

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