FMR1 and fragile X-associated tremor/ataxia syndrome: Both KI models, CGGdut and CGGnih, showed elevated Fmr1 mRNA levels, reduced Fmrp production, the presence of ubiquitin-positive intranuclear inclusions throughout the brain and specific behavioral deficits, including late-onset ataxia, memory impairment and impaired motor performance, all key features of FXTAS (Entezam et al., 2007; Willemsen et al., 2003; reviewed in Foote et al., 2016).