With the discovery that loss of function of the maternal copy of UBE3A gene causes AS, Jiang et al. created the Ube3amat−/pat+ KO mice, with a deletion on the maternally inherited Ube3a allele, which presents ataxia, inducible seizures, and sleep alterations, all features shown by AS individuals (Jiang et al., 1998; Colas et al., 2005; Dindot et al., 2007). The gene discussed is UBE3A; the disease is Ataxia.