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NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
Symbols NDUFS4 ; AQDQ; CI-18
External IDs OMIM602694 MGI1343135 HomoloGene1866 GeneCards: NDUFS4 Gene
RNA expression pattern
PBB GE NDUFS4 209303 at tn.png
More reference expression data
Species Human Mouse
Entrez 4724 17993
Ensembl ENSG00000164258 ENSMUSG00000021764
UniProt O43181 Q9CXZ1
RefSeq (mRNA) NM_002495 NM_010887
RefSeq (protein) NP_002486 NP_035017
Location (UCSC) Chr 5:
52.86 – 52.98 Mb
Chr 13:
114.29 – 114.39 Mb
PubMed search [1] [2]

NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial also known as NADH-ubiquinone oxidoreductase 18 kDa subunit is an enzyme that in humans is encoded by the NDUFS4 gene.[1][2][3]


Complex I, or NADH:ubiquinone oxidoreductase, the first multisubunit enzyme complex of the mitochondrial respiratory chain chain, plays a vital role in cellular ATP production, the primary source of energy for many crucial processes in living cells. It removes electrons from NADH and passes them by a series of different protein-coupled redox centers to the electron acceptor ubiquinone. In well-coupled mitochondria, the electron flux leads to ATP generation via the building of a proton gradient across the inner membrane. Complex I is composed of at least 41 subunits, of which 7 are encoded by the mitochondrial genome (ND1-6, ND4L) and the remainder by nuclear genes.[3][1]


  1. ^ a b van den Heuvel L, Ruitenbeek W, Smeets R, Gelman-Kohan Z, Elpeleg O, Loeffen J, Trijbels F, Mariman E, de Bruijn D, Smeitink J (Apr 1998). "Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit". Am J Hum Genet 62 (2): 262–8. doi:10.1086/301716. PMC 1376892. PMID 9463323. 
  2. ^ Emahazion T, Beskow A, Gyllensten U, Brookes AJ (Nov 1998). "Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chain". Cytogenet Cell Genet 82 (1–2): 115–9. doi:10.1159/000015082. PMID 9763677. 
  3. ^ a b "Entrez Gene: NDUFS4 NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)". 

Further reading[edit]