NADH dehydrogenase (ubiquinone) Fe-S protein 8 (NDUFS8) is a nuclear-encoded core subunit of human mitochondrial complex I. Defects in NDUFS8 are associated with Leigh syndrome and encephalomyopathy. This evidence concerns the gene NDUFS8 and mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria.