NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3
Also known as: NDUFB3
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)
- PF08122 NDUF_B12
- InterPro
- NDUFB3
- Functional cluster
- PLP-Dependent Aminotransferases & Lyases
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:0006120 mitochondrial electron transport, NADH to ubiquinone
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 25 MONDO:0032629
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 0.98
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 0.96
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 0.95
- ATP synthase subunit f, mitochondrial 0.72
- Uncharacterized protein DDB_G0276837 0.71
- ATP synthase subunit J, mitochondrial 0.70
- ATP synthase subunit f, mitochondrial 0.70
- ATP synthase subunit J, mitochondrial 0.70
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2 0.69
- ATP synthase subunit f, mitochondrial 0.68
Co-cited proteins · studied together in the literature
- Methionyl-tRNA formyltransferase, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 2 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial 2 shared papers
- Armadillo repeat-containing protein 6 1 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 1 shared papers
- 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
Literature · 11 cited papers
- The methyltransferase METTL9 mediates pervasive 1-methylhistidine modification in mammalian proteomes. Nat. Commun. · 2021
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- A recurrent mitochondrial p.Trp22Arg NDUFB3 variant causes a distinctive facial appearance, short stature and a mild biochemical and clinical phenotype. J. Med. Genet. · 2016
- N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
- Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing. J. Med. Genet. · 2012
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Generation and annotation of the DNA sequences of human chromosomes 2 and 4. Nature · 2005
- 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
- cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed. Biochem. Biophys. Res. Commun. · 1998
- Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits. Biochem. Biophys. Res. Commun. · 1997
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