NADH dehydrogenase [ubiquinone] 1 subunit C2
Also known as: NDUFC2
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis but required for the complex assembly. 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)
- PF06374 NDUF_C2
- InterPro
- NDUC2
- Functional cluster
- Photosystem II Reaction-Center & Antimicrobial Peptides
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 15
- GO:0035577 azurophil granule membrane
- GO:0005737 cytoplasm
- GO:0005743 mitochondrial inner membrane
- GO:0005739 mitochondrion
- GO:0005886 plasma membrane
- GO:0045271 respiratory chain complex I
- GO:0008137 NADH dehydrogenase (ubiquinone) activity
- GO:0009060 aerobic respiration
- GO:0006120 mitochondrial electron transport, NADH to ubiquinone
- GO:0032981 mitochondrial respiratory chain complex I assembly
- GO:1901223 negative regulation of non-canonical NF-kappaB signal transduction
- GO:1903427 negative regulation of reactive oxygen species biosynthetic process
- GO:2001171 positive regulation of ATP biosynthetic process
- GO:0010918 positive regulation of mitochondrial membrane potential
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 36 MONDO:0030902
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 subunit C2 1.00
- NADH dehydrogenase [ubiquinone] 1 subunit C2 1.00
- NADH dehydrogenase [ubiquinone] 1 subunit C2 0.99
- NADH dehydrogenase [ubiquinone] 1 subunit C2 0.97
- NADH dehydrogenase [ubiquinone] 1 subunit C2 0.96
- NADH dehydrogenase [ubiquinone] 1 subunit C2, isoform 2 0.96
- NADH dehydrogenase [ubiquinone] 1 subunit C2 0.92
- NADH-ubiquinone oxidoreductase 20.9 kDa subunit 0.74
- Ubiquinol-cytochrome c reductase complex assembly factor 5 0.70
- Ubiquinol-cytochrome c reductase complex assembly factor 5 0.67
- Cytochrome c oxidase subunit 6C 0.67
- Cytochrome c oxidase subunit 6C-1 0.66
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 2 shared papers
- Transmembrane protein 242 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 2 shared papers
- Complex I assembly factor TIMMDC1, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 2 shared papers
- Complex I assembly factor TMEM126B, mitochondrial 1 shared papers
- ATP synthase F(0) complex subunit C3, mitochondrial 1 shared papers
Literature · 11 cited papers
- TMEM70 and TMEM242 help to assemble the rotor ring of human ATP synthase and interact with assembly factors for complex I. Proc. Natl. Acad. Sci. U.S.A. · 2021
- Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I. EMBO Mol. Med. · 2020
- 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
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Human chromosome 11 DNA sequence and analysis including novel gene identification. 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
- Towards a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs. Genome Res. · 2001
- Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells. Genome Res. · 2000
- cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed. Biochem. Biophys. Res. Commun. · 1998
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