NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7
Also known as: NDUFB7
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)
- PF05676 NDUF_B7
- InterPro
- NDUB7
- Functional cluster
- RuBisCO & Carbon-Fixation Enzymes
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 8
- GO:0005743 mitochondrial inner membrane
- GO:0005758 mitochondrial intermembrane space
- 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 39 MONDO:0859320
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 0.98
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 0.98
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 0.88
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 0.86
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 0.84
- Uncharacterized protein DDB_G0275933 0.81
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 0.78
- COX assembly mitochondrial protein 0.77
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 0.77
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 2 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 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
Literature · 10 cited papers
- Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7. Hum. Mutat. · 2021
- 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
- NDUFB7 and NDUFA8 are located at the intermembrane surface of complex I. FEBS Lett. · 2011
- 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
- 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
- cDNA cloning of a novel cell adhesion protein expressed in human squamous carcinoma cells. Biochem. Biophys. Res. Commun. · 1990
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