MNs derived from the spinal cord of ALS patients and presymptomatic SOD1G93A mice show mutant SOD1 aggregates in intermembrane space, mitochondrial swelling, fragmentation of the mitochondrial network, peroxidation of mitochondrial membrane lipids, decreased coupling between mitochondrial oxidative phosphorylation and electron transport chain (ETC), and reduced expression of mitochondrial enzymes [195]. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.