The inhibition of mitochondrial fission by overexpression of inactivated Drp1 or Mfn2 prevents mitochondrial trafficking deficits in motor neurons expressing disease causing mutant SOD1 or TDP-43 [135,142], implying that, in addition to Miro1 deficiency, the mitochondrial fusion and fission dynamic abnormalities may also be responsible for impaired mitochondrial movement in ALS. This evidence concerns the gene RHOT1 and amyotrophic lateral sclerosis.