Changes in mitochondria morphology are one of the first hallmarks observed in motor neurons of ALS patients [106] and in cell or animal TDP-43 or SOD1-related models of ALS [107,108,109], together with the reduction in cellular respiration and ATP production, due to deficits in complex I, II, III, and IV activity that have been found in different models, i.e., the postmortem spinal cord of sporadic ALS patients or from skeletal muscle and lymphocytes derived from them [110]. Here, SOD1 is linked to amyotrophic lateral sclerosis.