NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial
Also known as: NDUFB8
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)
- PF05821 NDUF_B8
- InterPro
- NDUFB8, Ndufb8_metazoa
- 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:0005759 mitochondrial matrix
- 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 32 MONDO:0032635
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 0.98
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 0.97
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.75
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.72
- Probable NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.72
- Probable NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial 0.72
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 0.67
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 0.66
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 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 4 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 2 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 subunit C2 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 2 shared papers
- Acyl carrier protein, mitochondrial 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
Literature · 11 cited papers
- NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy. Am. J. Hum. Genet. · 2018
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- 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 DNA sequence and comparative analysis of human chromosome 10. Nature · 2004
- Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 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
- Identification of 167 polymorphisms in 88 genes from candidate neurodegeneration pathways. Gene · 1999
- 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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