In ALS patient muscle, peroxisome proliferator-activated receptor gamma coactivator 1-α (PGC-1α), a master regulator of normal mitochondrial function and biogenesis, is downregulated, leading to modifications in fatty acid signaling, and increased β-oxidation (Barroso et al., 2011; Thau et al., 2012). This evidence concerns the gene PPARGC1A and amyotrophic lateral sclerosis.