NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial

Function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity and assembly of complex I.

Classification

Family (Pfam)

PF12838 Fer4_7

InterPro

4Fe4S_Fe-S-bd, 4Fe4S_Fe_S_CS, NADH_quinone_OxRdtase_chainI

Functional cluster

Mixed Regulatory & Membrane Proteins

Experimental structures · PDB · 7

• 5XTB EM 3.40A
• 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 · 12

• GO:0005743 mitochondrial inner membrane
• GO:0005759 mitochondrial matrix
• GO:0005739 mitochondrion
• GO:0045271 respiratory chain complex I
• GO:0051539 4 iron, 4 sulfur cluster binding
• GO:0046872 metal ion binding
• GO:0008137 NADH dehydrogenase (ubiquinone) activity
• GO:0016651 oxidoreductase activity, acting on NAD(P)H
• 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 2 MONDO:0032606

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1.00
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1.00
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 1.00
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 0.99
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 0.99
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 0.97
• NADH dehydrogenase (ubiquinone) 23 kDa subunit 0.94
• NADH-ubiquinone oxidoreductase 23 kDa subunit, mitochondrial 0.92
• Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 0.89
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8-A, mitochondrial 0.88
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 0.87
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8-B, mitochondrial 0.87

Co-cited proteins · studied together in the literature

• Methionyl-tRNA formyltransferase, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 2 shared papers
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 1 shared papers
• Complex I assembly factor ACAD9, mitochondrial 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
• Parkinson disease protein 7 homolog 1 shared papers
• Neudesin 1 shared papers
• Serine/threonine-protein kinase PINK1, 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
• 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
• Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing. J. Med. Genet. · 2012
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• Sequence analysis of nuclear genes encoding functionally important complex I subunits in children with encephalomyopathy. J. Mol. Med. · 2005
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
• Late-onset Leigh syndrome in a patient with mitochondrial complex I NDUFS8 mutations. Neurology · 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 first nuclear-encoded complex I mutation in a patient with Leigh syndrome. Am. J. Hum. Genet. · 1998
• Genomic structure of the human NDUFS8 gene coding for the iron-sulfur TYKY subunit of the mitochondrial NADH:ubiquinone oxidoreductase. Gene · 1998
• … and 1 more in the literature graph