Three main mechanisms have been proposed to explain the pathogenesis of FXTAS, including the sequestration of CGG-binding proteins amplified by the elevated levels of FMR1 mRNA, the production of toxic FMRPolyG proteins due to RAN translation, and the chronic activation of the DNA damage response [7,8]. The gene discussed is FMR1; the disease is fragile X-associated tremor/ataxia syndrome.