Second, and most disconcerting, although inclusions in brain samples of patients with FXTAS contain the mutant FMR1 RNA with expanded CGG repeats [50], a mouse model, in which the endogenous eight CGG repeats of Fmr1 is replaced with an expansion containing around 100 CGG repeats, shows numerous ubiquitin inclusions but only rare aggregates of SAM68 or DROSHA-DGCR8, associated with rare RNA aggregates of expanded CGG repeats [60,67]. The gene discussed is DROSHA; the disease is fragile X-associated tremor/ataxia syndrome.