Yet although these unmethylated Fmr1hs341 mice experience molecular perturbations that closely resemble the FXTAS/FXPOI premutation (elevated Fmr1 transcript levels and reduced FMRP protein), they do not appear to exhibit any clear motor defects expected of FXTAS. The gene discussed is FMR1; the disease is fragile X-associated tremor/ataxia syndrome.