NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial
Also known as: NDUFA9, NDUFS2L
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Required for proper complex I assembly. 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)
- PF01370 Epimerase
- InterPro
- ComplexI_NDUFA9_subunit, Epimerase_deHydtase, NAD(P)-bd_dom_sf
- Functional cluster
- RuBisCO & Carbon-Fixation Enzymes
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 15
- GO:0005743 mitochondrial inner membrane
- GO:0005759 mitochondrial matrix
- GO:0031966 mitochondrial membrane
- GO:0005739 mitochondrion
- GO:0005634 nucleus
- GO:0045271 respiratory chain complex I
- GO:0008137 NADH dehydrogenase (ubiquinone) activity
- GO:0003954 NADH dehydrogenase activity
- GO:0044877 protein-containing complex binding
- GO:0009060 aerobic respiration
- GO:0007623 circadian rhythm
- GO:0006120 mitochondrial electron transport, NADH to ubiquinone
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
- GO:0006814 sodium ion transport
- GO:0006744 ubiquinone biosynthetic process
Disease associations
- mitochondrial complex I deficiency, nuclear type 26 MONDO:0032630
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 0.97
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 0.97
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 0.96
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 0.82
- NADH-ubiquinone oxidoreductase 40 kDa subunit, mitochondrial 0.78
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 0.69
- 3beta-hydroxysteroid dehydrogenase/Delta(5)-Delta(4) isomerase 1 0.60
- Short-chain dehydrogenase/reductase family 42E member 1 0.56
Co-cited proteins · studied together in the literature
- Tumor necrosis factor receptor superfamily member 3 1 shared papers
- Biogenesis of lysosome-related organelles complex 1 subunit 1 1 shared papers
- Biogenesis of lysosome-related organelles complex 1 subunit 1 1 shared papers
- ATP synthase F(1) complex subunit alpha, mitochondrial 1 shared papers
- Argininosuccinate synthase 1 shared papers
- Argininosuccinate synthase 1 shared papers
- Inosine-5'-monophosphate dehydrogenase 2 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 1 shared papers
- Neudesin 1 shared papers
- GEL complex subunit OPTI 1 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1 shared papers
Literature · 13 cited papers
- Rewiring of the Human Mitochondrial Interactome during Neuronal Reprogramming Reveals Regulators of the Respirasome and Neurogenesis. IScience · 2019
- CLOCK acetylates ASS1 to drive circadian rhythm of ureagenesis. Mol. Cell · 2017
- NDUFA9 point mutations cause a variable mitochondrial complex I assembly defect. Clin. Genet. · 2018
- 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
- Identification of a molecular component of the mitochondrial acetyl transferase program; a novel role for GCN5L1. Biochem. J. · 2012
- Defective NDUFA9 as a novel cause of neonatally fatal complex I disease. J. Med. Genet. · 2012
- 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
- Construction and evaluation of a hncDNA library of human 12p transcribed sequences derived from a somatic cell hybrid. Genomics · 1993
- … and 1 more in the literature graph
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