This murine model recapitulates some of the molecular, histological, and neurobehavioral deficits observed in premutation carriers (i.e., elevated FMR1 mRNA, FMRP protein levels reduced or normal), and with age they develop intranuclear inclusions in neurons and astrocytes, ataxia-like mild motor dysfunctions, anxiety, and cognitive impairments (Hunsaker et al., 2009, 2011, 2012; Wenzel et al., 2010). Here, FMR1 is linked to Ataxia.