NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12
Also known as: DAP13, NDUFA12
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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.
Classification
- Family (Pfam)
- PF05071 NDUFA12
- InterPro
- NDUFA12
- Functional cluster
- Oxidoreductases & Polyketide Synthases
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 7
- GO:0005743 mitochondrial inner membrane
- GO:0005739 mitochondrion
- GO:0045271 respiratory chain complex I
- GO:0008137 NADH dehydrogenase (ubiquinone) activity
- GO:0009060 aerobic respiration
- GO:0042775 mitochondrial ATP synthesis coupled electron transport
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 23 MONDO:0032627
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 0.99
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 0.99
- Probable NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 0.92
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit N7BM 0.91
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 0.88
- Probable NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 0.82
- Uncharacterized protein RP725 0.70
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 0.66
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 0.64
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 0.61
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 1 shared papers
- Distal membrane-arm assembly complex protein 1 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 1 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial 1 shared papers
- Distal membrane-arm assembly complex protein 2 1 shared papers
Literature · 11 cited papers
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB. Proc. Natl. Acad. Sci. U.S.A. · 2012
- Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome. J. Med. Genet. · 2011
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- The finished DNA sequence of human chromosome 12. Nature · 2006
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
- Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning. Proc. Natl. Acad. Sci. U.S.A. · 2000
- Characterization of the human complex I NDUFB7 and 17.2-kDa cDNAs and mutational analysis of 19 genes of the HP fraction in complex I-deficient-patients. Hum. Genet. · 2000
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