There is increasing interest in the role of the peroxisome proliferator-activated receptor γ coactivator 1 α (PGC1α) in the brain, given mounting evidence that its levels are modulated in various neurodegenerative disorders including Huntington’s (HD), Parkinson’s (PD), and Alzheimer’s disease (AD) as well as amyotrophic lateral sclerosis (Katsouri et al, 2012; Dumont et al, 2014). This evidence concerns the gene PPARGC1A and Huntington disease.