It has long been shown that mitochondria became fragmented concurrent with the changed expression of several mitochondrial fusion and fission regulators such as Drp1, OPA1, Mfn1, and Fis1 in experimental models expressing ALS-associated mutant SOD1, well preceding motor neuronal loss and symptom onset [134,135,136,137,138,139]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.