Hmdb loader
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
HMDB Protein ID HMDBP00182
Secondary Accession Numbers
  • 5414
  • HMDBP03448
Name NADH-ubiquinone oxidoreductase chain 3
  1. NADH dehydrogenase subunit 3
Gene Name MT-ND3
Protein Type Unknown
Biological Properties
General Function Involved in NADH dehydrogenase (ubiquinone) activity
Specific Function Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity).
  • Oxidative phosphorylation
  • Parkinson disease
NADH + Coenzyme Q10 → NAD + QH(2) details
QH2 + Acceptor → Ubiquinone-2 + Reduced acceptor details
GO Classification
Biological Process
small molecule metabolic process
response to light intensity
mitochondrial electron transport, NADH to ubiquinone
response to hormone stimulus
response to oxidative stress
Cellular Component
mitochondrial respiratory chain complex I
integral to membrane
catalytic activity
nadh dehydrogenase activity
nadh dehydrogenase (quinone) activity
nadh dehydrogenase (ubiquinone) activity
oxidoreductase activity
oxidoreductase activity, acting on nadh or nadph
Molecular Function
NADH dehydrogenase (ubiquinone) activity
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion membrane
  2. Multi-pass membrane protein
Gene Properties
Chromosome Location Not Available
Locus Not Available
Gene Sequence
>346 bp
Protein Properties
Number of Residues 115
Molecular Weight 13185.87
Theoretical pI 4.41
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>NADH-ubiquinone oxidoreductase chain 3
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P03897
UniProtKB/Swiss-Prot Entry Name NU3M_HUMAN
PDB IDs Not Available
GenBank Gene ID J01415
GeneCard ID MT-ND3
GenAtlas ID MT-ND3
General References
  1. Murray J, Zhang B, Taylor SW, Oglesbee D, Fahy E, Marusich MF, Ghosh SS, Capaldi RA: The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J Biol Chem. 2003 Apr 18;278(16):13619-22. Epub 2003 Feb 28. [PubMed:12611891 ]
  2. Anderson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG: Sequence and organization of the human mitochondrial genome. Nature. 1981 Apr 9;290(5806):457-65. [PubMed:7219534 ]
  3. Moilanen JS, Finnila S, Majamaa K: Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J. Mol Biol Evol. 2003 Dec;20(12):2132-42. Epub 2003 Aug 29. [PubMed:12949126 ]
  4. Ingman M, Kaessmann H, Paabo S, Gyllensten U: Mitochondrial genome variation and the origin of modern humans. Nature. 2000 Dec 7;408(6813):708-13. [PubMed:11130070 ]
  5. Ingman M, Gyllensten U: Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines. Genome Res. 2003 Jul;13(7):1600-6. [PubMed:12840039 ]
  6. Coble MD, Just RS, O'Callaghan JE, Letmanyi IH, Peterson CT, Irwin JA, Parsons TJ: Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians. Int J Legal Med. 2004 Jun;118(3):137-46. Epub 2004 Feb 4. [PubMed:14760490 ]
  7. Chomyn A, Mariottini P, Cleeter MW, Ragan CI, Matsuno-Yagi A, Hatefi Y, Doolittle RF, Attardi G: Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase. Nature. 1985 Apr 18-24;314(6012):592-7. [PubMed:3921850 ]
  8. Marzuki S, Noer AS, Lertrit P, Thyagarajan D, Kapsa R, Utthanaphol P, Byrne E: Normal variants of human mitochondrial DNA and translation products: the building of a reference data base. Hum Genet. 1991 Dec;88(2):139-45. [PubMed:1757091 ]
  9. Oliver NA, Greenberg BD, Wallace DC: Assignment of a polymorphic polypeptide to the human mitochondrial DNA unidentified reading frame 3 gene by a new peptide mapping strategy. J Biol Chem. 1983 May 10;258(9):5834-9. [PubMed:6343397 ]
  10. Taylor RW, Singh-Kler R, Hayes CM, Smith PE, Turnbull DM: Progressive mitochondrial disease resulting from a novel missense mutation in the mitochondrial DNA ND3 gene. Ann Neurol. 2001 Jul;50(1):104-7. [PubMed:11456298 ]
  11. Sarzi E, Brown MD, Lebon S, Chretien D, Munnich A, Rotig A, Procaccio V: A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia. Am J Med Genet A. 2007 Jan 1;143A(1):33-41. [PubMed:17152068 ]