NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6
Also known as: LYRM6, NADHB14, NDUFA6
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not 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)
- PF13233 Complex1_LYR_2
- InterPro
- Complex1_LYR_NDUFA6_LYRM6, NADH_Ub_cplx-1_asu_su-6
- Functional cluster
- Central Metabolic Enzymes (KARI/Glycolysis)
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 9
- 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:0032981 mitochondrial respiratory chain complex I assembly
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 33 MONDO:0032636
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 0.98
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 0.96
- NADH-ubiquinone oxidoreductase 14.8 kDa subunit 0.84
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 0.78
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 0.75
- LYR motif-containing protein 4 homolog 0.70
- Protein ISD11 0.69
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 0.69
- LYR motif-containing protein 4A 0.69
Co-cited proteins · studied together in the literature
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial 2 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 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
Literature · 11 cited papers
- Bi-allelic mutations in NDUFA6 establish its role in early-onset isolated mitochondrial complex I deficiency. Am. J. Hum. 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
- Toward a comprehensive characterization of a human cancer cell phosphoproteome. J. Proteome Res. · 2013
- 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
- A genome annotation-driven approach to cloning the human ORFeome. Genome Biol. · 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
- The DNA sequence of human chromosome 22. Nature · 1999
- Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits. Biochem. Biophys. Res. Commun. · 1997
A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.