This gene encodes an nuclear-encoded accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I, or NADH:ubiquinone oxidoreductase). Complex I removes electrons from NADH and passes them to the electron acceptor ubiquinone. Mutations in this gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015]
Alternative Names
CI-18 kDa CI-18(IP) CI-AQDQ complex I 18kDa subunit complex I-18 kDa complex I-AQDQ mitochondrial respiratory chain complex I (18-KD subunit) NADH dehydrogenase (ubiquinone) Fe-S protein 4 18kDa (NADH-coenzyme Q reductase) isoform NDUFS4_1 NADH dehydrogenase (ubiquinone) Fe-S protein 4 18kDa (NADH-coenzyme Q reductase) isoform NDUFS4_2 NADH dehydrogenase (ubiquinone) Fe-S protein 4 18kDa (NADH-coenzyme Q reductase) NADH-ubiquinone oxidoreductase 18 kDa subunit subunit of Mitochondrial Complex I