Similarly, the sequestration of Drosha and DiGeorge syndrome critical region 8 complex (Drosha-DGCR8) is involved in the processing of miRNA precursors in the nucleus (Sellier et al., 2013) and has been linked to the reduced generation of mature miRNAs in the brains of FXTAS patients. The gene discussed is DROSHA; the disease is fragile X-associated tremor/ataxia syndrome.