A number of reports have been published describing certain aspects of locomotor alterations in PGC-1α-deficient mice, and recently, comprehensive works have together circumscribed a motor phenotype of decreased locomotion, muscle weakness, and ataxia, in association with the absence of either PGC-1α or FL-PGC-1α in systemic and CNS-specific knockouts (Lucas et al., 2012, 2014b; Szalardy et al., 2016a). This evidence concerns the gene PPARGC1A and Ataxia.