NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, 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)

PF00329 Complex1_30kDa

InterPro

NADH_DH_suC, NADH_quin_OxRdtase_su_C/D-like, NADH_UbQ_OxRdtase_30kDa_su, NADH_UbQ_OxRdtase_CS

Functional cluster

Viral Structural & Replication 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 · 14

• GO:0005743 mitochondrial inner membrane
• GO:0005759 mitochondrial matrix
• GO:0031966 mitochondrial membrane
• GO:0005739 mitochondrion
• GO:0045271 respiratory chain complex I
• GO:0009055 electron transfer activity
• GO:0008137 NADH dehydrogenase (ubiquinone) activity
• GO:0003954 NADH dehydrogenase 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
• GO:0072593 reactive oxygen species metabolic process
• GO:0021762 substantia nigra development

Disease associations

• mitochondrial complex I deficiency, nuclear type 8 MONDO:0032613

Drugs targeting this protein · 2

• METFORMIN HYDROCHLORIDE inhibitor
• ME-344 inhibitor

Related proteins · sequence + function similarity

• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.98
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.94
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.92
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.83
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.77
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 0.73
• ORFB polyprotein 0.60
• Probable NADH-ubiquinone oxidoreductase 30.4 kDa subunit, mitochondrial 0.60
• Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial 0.60
• UPF0598 protein CG30010 0.57
• Envelope glycoprotein L 0.57
• D-aminoacyl-tRNA deacylase 2 0.57

Co-cited proteins · studied together in the literature

• NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 1 shared papers
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial 2 shared papers
• Methionyl-tRNA formyltransferase, mitochondrial 1 shared papers
• NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 2 shared papers
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 1 shared papers
• NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 1 shared papers
• Complex I assembly factor ACAD9, mitochondrial 1 shared papers
• Ribosomal protein uL24-like 1 shared papers
• Thioredoxin, 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

Literature · 19 cited papers

• Rewiring of the Human Mitochondrial Interactome during Neuronal Reprogramming Reveals Regulators of the Respirasome and Neurogenesis. IScience · 2019
• A Novel NDUFS3 mutation in a Chinese patient with severe Leigh syndrome. J. Hum. Genet. · 2018
• 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
• Human mitochondrial NDUFS3 protein bearing Leigh syndrome mutation is more prone to aggregation than its wild-type. Biochimie · 2013
• 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
• Global profiling of protease cleavage sites by chemoselective labeling of protein N-termini. Proc. Natl. Acad. Sci. U.S.A. · 2009
• Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease. Am. J. Hum. Genet. · 2009
• Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells. J. Biol. Chem. · 2008
• Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits. J. Biol. Chem. · 2007
• Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature · 2006
• … and 7 more in the literature graph