NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, 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 of complex I.

Classification

Family (Pfam)

PF01058 Oxidored_q6

InterPro

NADH_UbQ_OxRdtase-like_20kDa, NADH_UQ_OxRdtase_20Kd_su

Functional cluster

Ribosomal Protein S5 & Biosynthetic Enzymes

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 · 17

• GO:0005743 mitochondrial inner membrane
• GO:0005759 mitochondrial matrix
• GO:0005739 mitochondrion
• GO:0043025 neuronal cell body
• GO:0045271 respiratory chain complex I
• GO:0097060 synaptic membrane
• GO:0051539 4 iron, 4 sulfur cluster binding
• GO:0046872 metal ion binding
• GO:0008137 NADH dehydrogenase (ubiquinone) activity
• GO:0016655 oxidoreductase activity, acting on NAD(P)H, quinone or similar compound as acceptor
• GO:0002020 protease binding
• GO:0048038 quinone binding
• GO:0009060 aerobic respiration
• GO:0015990 electron transport coupled proton transport
• 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 3 MONDO:0032608

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 1.00
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.99
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.99
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.98
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.93
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.92
• Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.92
• Probable NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 0.91
• NADH-quinone oxidoreductase subunit B 0.88
• NADH-quinone oxidoreductase subunit B 0.88
• NADH-quinone oxidoreductase subunit B 0.87
• NADH-quinone oxidoreductase subunit B 0.87

Co-cited proteins · studied together in the literature

• Arginine-hydroxylase NDUFAF5, mitochondrial 1 shared papers

Literature · 11 cited papers

• NDUFAF5 hydroxylates NDUFS7 at an early stage in the assembly of human complex I. J. Biol. Chem. · 2016
• N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
• Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
• A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome. Mol. Genet. Metab. · 2007
• 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
• The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
• Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I. Ann. Neurol. · 1999
• Human mitochondrial complex I in health and disease. Am. J. Hum. Genet. · 1999
• Assignment of the PSST subunit gene of human mitochondrial complex I to chromosome 19p13. Genomics · 1996