Like PGC-1α, mitochondrial OXPHOS dysfunction occurs in a variety of neurodegenerative diseases, including FRDA (Coppola et al., 2009; Johri et al., 2013; Lopez-Gallardo et al., 2011; Mastroeni et al., 2016; McGill and Beal, 2006; Pesini et al., 2014; Salehi et al., 2014; Seelert et al., 2009; Shin et al., 2011; Sweeney and Song, 2016; Thau et al., 2012). This evidence concerns the gene FXN and neurodegenerative disease.