NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial
Also known as: NDUFV2
Function
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor (Probable). Parts of the peripheral arm of the enzyme, where the electrons from NADH are accepted by flavin mononucleotide (FMN) and then passed along a chain of iron-sulfur clusters by electron tunnelling to the final acceptor ubiquinone (Probable). Contains one iron-sulfur cluster (Probable).
Classification
- Family (Pfam)
- PF01257 2Fe-2S_thioredx
- InterPro
- NuoE-like, NuoE_dom, NuoE_N, Thioredoxin-like_sf
- Functional cluster
- Oxidoreductases & Polyketide Synthases
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 13
- GO:0005743 mitochondrial inner membrane
- GO:0005739 mitochondrion
- GO:0045271 respiratory chain complex I
- GO:0051537 2 iron, 2 sulfur cluster binding
- GO:0009055 electron transfer activity
- GO:0046872 metal ion binding
- GO:0008137 NADH dehydrogenase (ubiquinone) activity
- GO:0016491 oxidoreductase activity
- GO:0009060 aerobic respiration
- GO:0048738 cardiac muscle tissue development
- GO:0006120 mitochondrial electron transport, NADH to ubiquinone
- GO:0007399 nervous system development
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 7 MONDO:0032612
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.98
- Probable NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.93
- NADH-ubiquinone oxidoreductase 24 kDa subunit, mitochondrial 0.90
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.88
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 0.85
- NADH-quinone oxidoreductase subunit E 0.71
- NADH-quinone oxidoreductase subunit E 0.70
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial 1 shared papers
- Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 1 shared papers
- Cytochrome b-c1 complex subunit 7 1 shared papers
- Cytochrome b-c1 complex subunit 8 1 shared papers
- Cytochrome c1, heme protein, mitochondrial 1 shared papers
- Cytochrome b-c1 complex subunit 9 1 shared papers
- Cytochrome c oxidase subunit 6B1 1 shared papers
- Cytochrome b-c1 complex subunit 6, mitochondrial 1 shared papers
- Cytochrome b-c1 complex subunit 10 1 shared papers
- Cytochrome b-c1 complex subunit Rieske, mitochondrial 1 shared papers
- Cytochrome c oxidase subunit 8A, mitochondrial 1 shared papers
- Cytochrome c oxidase subunit 7B, mitochondrial 1 shared papers
Literature · 10 cited papers
- Architecture of human mitochondrial respiratory megacomplex I2III2IV2. Cell · 2017
- Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome. Eur. J. Paediatr. Neurol. · 2015
- 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
- Mitochondrial c-Src regulates cell survival through phosphorylation of respiratory chain components. Biochem. J. · 2012
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy. Hum. Mutat. · 2003
- The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
- Mitochondrial NADH-ubiquinone reductase: complementary DNA sequences of import precursors of the bovine and human 24-kDa subunit. Biochemistry · 1989
A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.