Compared to SOD1 WT, ALS mutant SOD1 G93A significantly reduced the level of endogenous Miro1 in cortical neurons indicating that the ALS mutant SOD1-associated impairment of mitochondrial axonal transport may be due to reduced Miro1 levels (Fig. 3B). This evidence concerns the gene RHOT1 and amyotrophic lateral sclerosis.