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NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
Symbols NDUFS1 ; CI-75Kd; CI-75k; PRO1304
External IDs OMIM157655 MGI2443241 HomoloGene3670 GeneCards: NDUFS1 Gene
EC number,
RNA expression pattern
PBB GE NDUFS1 203039 s at tn.png
More reference expression data
Species Human Mouse
Entrez 4719 227197
Ensembl ENSG00000023228 ENSMUSG00000025968
UniProt P28331 Q91VD9
RefSeq (mRNA) NM_001199981 NM_001160038
RefSeq (protein) NP_001186910 NP_001153510
Location (UCSC) Chr 2:
206.98 – 207.02 Mb
Chr 1:
63.14 – 63.18 Mb
PubMed search [1] [2]

NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial is an enzyme that in humans is encoded by the NDUFS1 gene.[1][2]


The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.[2]

Clinical significance[edit]

Mutations in this gene are associated with complex I deficiency.[2]


  1. ^ Chow W, Ragan I, Robinson BH (Nov 1991). "Determination of the cDNA sequence for the human mitochondrial 75-kDa Fe-S protein of NADH-coenzyme Q reductase". Eur J Biochem 201 (3): 547–50. doi:10.1111/j.1432-1033.1991.tb16313.x. PMID 1935949. 
  2. ^ a b c "Entrez Gene: NDUFS1 NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)". 

Further reading[edit]