Impairments in PGC-1α-mediated gene networks are observed in human diseases, including neurodegenerative disorders exemplified by Huntington’s disease (St-Pierre et al., 2006; Soyal et al., 2012; McMeekin et al., 2018) and Parkinson’s disease (Zheng et al., 2010; Clark et al., 2011; Mudò et al., 2012; Jiang et al., 2016; Mäkelä et al., 2016b). Here, PPARGC1A is linked to juvenile Huntington disease.