NADH-ubiquinone oxidoreductase chain 3

UniProt P03897 Organism Homo sapiens Gene MT-ND3, MTND3, NADH3, ND3 EC 7.1.1.2

Also known as: MT-ND3, MTND3, NADH3, ND3

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): PF00507 Oxidored_q4
InterPro: NADH_UbQ/plastoQ_OxRdtase_su3, NDAH_ubi_oxred_su3_sf
Functional cluster: Cytochrome b & NADH Dehydrogenase Subunits

Experimental structures · PDB · 5

5XTC EM 3.70A
5XTD EM 3.70A
9CWT EM 3.44A
9I4I EM 2.63A
9TI4 EM 2.66A

A predicted model is available from AlphaFold.

Gene Ontology · 10

Disease associations

Leigh syndrome MONDO:0009723
mitochondrial complex I deficiency, mitochondrial type 1 MONDO:0027068

Drugs targeting this protein · 2

METFORMIN HYDROCHLORIDE inhibitor
ME-344 inhibitor

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

NADH-ubiquinone oxidoreductase chain 5 8 shared papers
NADH-ubiquinone oxidoreductase chain 4 8 shared papers
NADH-ubiquinone oxidoreductase chain 4L 7 shared papers
NADH-ubiquinone oxidoreductase chain 2 8 shared papers
NADH-ubiquinone oxidoreductase chain 1 7 shared papers
Iron-sulfur cluster transfer protein NUBPL 1 shared papers
NADH-ubiquinone oxidoreductase chain 6 6 shared papers
ATP synthase F(0) complex subunit 8 6 shared papers
Cytochrome c oxidase subunit 1 6 shared papers
Complex I assembly factor TMEM126B, mitochondrial 2 shared papers
ATP synthase F(0) complex subunit a 6 shared papers
FAD-dependent oxidoreductase domain-containing protein 1 1 shared papers

Literature · 17 cited papers

TMEM70 and TMEM242 help to assemble the rotor ring of human ATP synthase and interact with assembly factors for complex I. Proc. Natl. Acad. Sci. U.S.A. · 2021
Dissecting the Roles of Mitochondrial Complex I Intermediate Assembly Complex Factors in the Biogenesis of Complex I. Cell Rep. · 2020
Rapid identification of a novel complex I MT-ND3 m.10134C>A mutation in a Leigh syndrome patient. PLoS ONE · 2014
Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency. Nat. Genet. · 2010
A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia. Am. J. Med. Genet. A · 2007
Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians. Int. J. Legal Med. · 2004
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency. Ann. Neurol. · 2004
Lineage-specific selection in human mtDNA: lack of polymorphisms in a segment of MTND5 gene in haplogroup J. Mol. Biol. Evol. · 2003
Mitochondrial genome variation and evolutionary history of Australian and New Guinean aborigines. Genome Res. · 2003
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
Progressive mitochondrial disease resulting from a novel missense mutation in the mitochondrial DNA ND3 gene. Ann. Neurol. · 2001
… and 5 more in the literature graph

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