Various RBPs have been reported in human, fly and mouse FXTAS models, such as Pur-alpha (Pura), Heterogeneous nuclear ribonucleoproteins A2/B1 (hnRNPA2/B1), Muscleblind Like Splicing Regulator 1 (MBNL1), SRC associated in mitosis of 68 kDa (Sam68), CUG-binding proteins (CUGBP1), Heterogeneous nuclear ribonuclearproteins (hnRNP G), and Drosha and DiGeorge syndrome critical region 8 complex (Drosha-DGCR8; Jin et al., 2007; Sofola et al., 2007; Sellier et al., 2010, 2013; Muslimov et al., 2011). This evidence concerns the gene DGCR8 and fragile X-associated tremor/ataxia syndrome.