Interestingly, in contrast to FXS, in both FXPOI and FXTAS, expression of FMR1 protein is not or only mildly decreased, and increased transcription of repeat-containing FMR1 mRNA sequestering RBPs and/or being translated into aberrant FMR1 protein may represent the dominating pathogenic mechanism [26, 27]. The gene discussed is FMR1; the disease is fragile X-associated tremor/ataxia syndrome.