NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1
Also known as: NDUFA1
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)
- PF15879 MWFE
- InterPro
- NADH_Ub_cplx-1_asu_su-1
- Functional cluster
- Membrane Transporters & GPCRs
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 8
- GO:0005743 mitochondrial inner membrane
- GO:0031966 mitochondrial 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 12 MONDO:0026720
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 0.98
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 0.97
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 0.97
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 0.96
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 0.79
- Uncharacterized transmembrane protein SSD449 0.75
- Uncharacterized protein PB8E5.10 0.71
- Non-classical export protein 1 0.70
- Cytochrome b-c1 complex subunit 8 0.69
- Cytochrome b-c1 complex subunit 8 0.69
Co-cited proteins · studied together in the literature
- NF-kappa-B-repressing factor 1 shared papers
- E3 ubiquitin-protein ligase RNF113A 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] iron-sulfur protein 6, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial 1 shared papers
Literature · 7 cited papers
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy. Ann. Neurol. · 2007
- The consensus coding sequences of human breast and colorectal cancers. Science · 2006
- 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
- Identification of a new member (ZNF183) of the Ring finger gene family in Xq24-25. Gene · 1997
- Isolation, mapping, and genomic structure of an X-linked gene for a subunit of human mitochondrial complex I. Genomics · 1996
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