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Identification |
HMDB Protein ID
| HMDBP00182 |
Secondary Accession Numbers
| |
Name
| NADH-ubiquinone oxidoreductase chain 3 |
Synonyms
|
- 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).
|
Pathways
|
- Oxidative phosphorylation
- Parkinson disease
|
Reactions
|
NADH + Coenzyme Q10 → NAD + QH(2) |
details
|
QH2 + Acceptor → Ubiquinone-2 + Reduced acceptor |
details
|
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GO Classification
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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 |
Function |
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 |
Process |
metabolic process |
oxidation reduction |
|
Cellular Location
|
- Mitochondrion membrane
- Multi-pass membrane protein
|
Gene Properties |
Chromosome Location
| Not Available |
Locus
| Not Available |
SNPs
| MT-ND3 |
Gene Sequence
|
>346 bp
ATAAACTTCGCCTTAATTTTAATAATCAACACCCTCCTAGCCTTACTACTAATAATTATT
ACATTTTGACTACCACAACTCAACGGCTACATAGAAAAATCCACCCCTTACGAGTGCGGC
TTCGACCCTATATCCCCCGCCCGCGTCCCTTTCTCCATAAAATTCTTCTTAGTAGCTATT
ACCTTCTTATTATTTGATCTAGAAATTGCCCTCCTTTTACCCCTACCATGAGCCCTACAA
ACAACTAACCTGCCACTAATAGTTATGTCATCCCTCTTATTAATCATCATCCTAGCCCTA
AGTCTGGCCTATGAGTGACTACAAAAAGGATTAGACTGAACCGAAT
|
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
MNFALILMINTLLALLLMIITFWLPQLNGYMEKSTPYECGFDPMSPARVPFSMKFFLVAI
TFLLFDLEIALLLPLPWALQTTNLPLMVMSSLLLIIILALSLAYEWLQKGLDWTE
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External Links |
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 |
HGNC ID
| HGNC:7458 |
References |
General References
| - 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
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