In terms of potential molecular factors regulating skeletal muscle mitochondria in ALS, muscle samples from patients with FALS and SALS, as well as the SOD1G93A ALS mouse model, exhibit a significant reduction in the mRNA and protein levels of the transcriptional co-activator, peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α), when compared with age matched control subjects and nerve disease (ND) control patients (Russell et al., 2012; Thau et al., 2012). The gene discussed is PPARGC1A; the disease is amyotrophic lateral sclerosis.