NADH-ubiquinone oxidoreductase chain 4

UniProt P03905 Organism Homo sapiens Gene MT-ND4, MTND4, NADH4, ND4 EC 7.1.1.2

Also known as: MT-ND4, MTND4, NADH4, ND4

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): PF01059 Oxidored_q5_N, PF00361 Proton_antipo_M
InterPro: NADH4_N, NADH_Q_OxRdtase_chainM/4, NADH_UbQ_OxRdtase, ND/Mrp_TM
Functional cluster: Cytochrome b & NADH Dehydrogenase Subunits

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 · 15

Disease associations

Leber optic atrophy and dystonia MONDO:0010772
Leber hereditary optic neuropathy MONDO:0010788
MELAS syndrome MONDO:0010789

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 6 8 shared papers
ATP synthase F(0) complex subunit a 8 shared papers
NADH-ubiquinone oxidoreductase chain 5 10 shared papers
NADH-ubiquinone oxidoreductase chain 1 8 shared papers
NADH-ubiquinone oxidoreductase chain 3 8 shared papers
Cytochrome b 4 shared papers
NADH-ubiquinone oxidoreductase chain 4L 7 shared papers
NADH-ubiquinone oxidoreductase chain 2 7 shared papers
Iron-sulfur cluster transfer protein NUBPL 1 shared papers
ATP synthase F(0) complex subunit 8 6 shared papers
Cytochrome c oxidase subunit 1 6 shared papers
FAD-dependent oxidoreductase domain-containing protein 1 1 shared papers

Literature · 22 cited papers

N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
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
Structural organization of mitochondrial human complex I: role of the ND4 and ND5 mitochondria-encoded subunits and interaction with prohibitin. Biochem. J. · 2004
Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians. Int. J. Legal Med. · 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
Mitochondrial genome diversity of native Americans supports a single early entry of founder populations into America. Am. J. Hum. Genet. · 2002
Mitochondrial genome variation and the origin of modern humans. Nature · 2000
Leber's hereditary optic neuropathy in Indonesia: two families with the mtDNA 11778G>A and 14484T>C mutations. Hum. Mutat. Suppl. · 1998
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia. Am. J. Hum. Genet. · 1996
… and 10 more in the literature graph

A document in the lmmol reference corpus — open public data (UniProt, GO, PDB, the literature graph) rendered as a single cross-linked page. Hover any link to preview its target.