NDUFS8 contains two tetranuclear iron-sulfur clusters (N6a and N6b) and plays a key role in electron transfer in complex I. NDUFS8 deficiency caused by missense mutations in NDUFS8 gene was found as the first molecular genetic link between a nuclear-encoded subunit of complex I and Leigh syndrome, which is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in the subcortical areas of the brain [13]. Here, NDUFS8 is linked to nervous system disorder.