In individuals carrying premutation alleles the FMR1 mRNA levels are increased and progressively accumulates in inclusions (Tassone et al., 2004; Arocena et al., 2005) that result in the fragile X-associated Tremor Ataxia syndrome (FXTAS) (MIM# 300623), a late onset autonomic disorder with cognitive dysfunction (Jacquemont et al., 2004). The gene discussed is FMR1; the disease is fragile X-associated tremor/ataxia syndrome.