NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
Also known as: NDUFS6
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)
- PF10276 zf-CHCC
- InterPro
- NDUFS6, Znf_CHCC
- Functional cluster
- Membrane Channels & Lipid-Anchored Proteins
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 8
- GO:0005743 mitochondrial inner membrane
- GO:0005739 mitochondrion
- GO:0045271 respiratory chain complex I
- GO:0009055 electron transfer activity
- 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 9 MONDO:0032615
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.99
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.97
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.96
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.95
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.93
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 0.63
- Small ribosomal subunit protein bS18m 0.53
- Cytochrome c oxidase subunit 5b-2, mitochondrial 0.52
- Protein FAM162B 0.52
- Large ribosomal subunit protein bL32m 0.52
- Protein FAM162B 0.52
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial 1 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] 1 beta subcomplex subunit 5, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial 1 shared papers
- Distal membrane-arm assembly complex protein 2 1 shared papers
Literature · 9 cited papers
- 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
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews. Eur. J. Hum. Genet. · 2009
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency. J. Clin. Invest. · 2004
- The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
- cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed. Biochem. Biophys. Res. Commun. · 1998
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