NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11

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

InterPro

NDUFA11

Functional cluster

Membrane Transporters & GPCRs

Experimental structures · PDB · 7

• 5XTC EM 3.70A
• 5XTD EM 3.70A
• 5XTH EM 3.90A
• 5XTI EM 17.40A
• 9CWT EM 3.44A
• 9I4I EM 2.63A
• 9TI4 EM 2.66A

A predicted model is available from AlphaFold.

Gene Ontology · 6

• GO:0005743 mitochondrial inner membrane
• GO:0005739 mitochondrion
• GO:0045271 respiratory chain complex I
• 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 14 MONDO:0032619

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 1.00
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 1.00
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 0.98
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 0.97
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 0.97
• Outer envelope pore protein 16-3, chloroplastic/mitochondrial 0.81
• NADH-ubiquinone oxidoreductase 21.3 kDa subunit 0.78
• Mitochondrial import inner membrane translocase subunit TIM23-3 0.77
• Mitochondrial import inner membrane translocase subunit TIM23-2 0.75
• Mitochondrial import inner membrane translocase subunit TIM23-1 0.72
• Mitochondrial import inner membrane translocase subunit TIM22 0.72
• Mitochondrial import inner membrane translocase subunit TIM22 0.72

Co-cited proteins · studied together in the literature

• 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 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
• Distal membrane-arm assembly complex protein 2 1 shared papers
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 1 shared papers
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 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
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation. Ann. Neurol. · 2008
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• The DNA sequence and biology of human chromosome 19. Nature · 2004
• Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
• Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides. Nat. Biotechnol. · 2003
• The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003