For the roles of CHCHD10 in the nucleus, opposite results were reported in another study, where both wild-type CHCHD10 and ALS-FTD-associated mutants activated nucleus-encoded mitochondrial complex I genes, NADH dehydrogenase iron-sulfur protein 3 and NADH dehydrogenase-1ß subcomplex subunit 6 [52]. Here, CHCHD10 is linked to amyotrophic lateral sclerosis.