Fragile-X tremor and ataxia syndrome (FXTAS), a Fragile-X mental retardation (FMR1) gene-related-phenotype, results from a pre-mutation condition with 55–200 200 CGG trinucleotide repeats and is characterized by paradoxically increased mRNA expression of FMR1 [55]. Here, FMR1 is linked to fragile X-associated tremor/ataxia syndrome.