CHCHD10 and amyotrophic lateral sclerosis: Specifically, we found that C. elegans deficient in har-1, the closest orthologue to human CHCHD10, display severe abnormalities in locomotion and reduced lifespan associated with mitochondrial superoxide overproduction, which were completely rescued by WT CHCHD10 but not FTD/ALS-associated CHCHD10 mutations (R15L and S59L).