NADH-ubiquinone oxidoreductase chain 5

UniProt P03915 Organism Homo sapiens Gene MT-ND5, MTND5, NADH5, ND5 EC 7.1.1.2

Also known as: MT-ND5, MTND5, NADH5, ND5

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): PF06455 NADH5_C, PF00361 Proton_antipo_M, PF00662 Proton_antipo_N
InterPro: NADH_DH_su5_C, NADHpl_OxRdtase_5_subgr, ND/Mrp_TM, NU5C-like, Proton_antipo_N
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 · 11

Disease associations

Leigh syndrome MONDO:0009723
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 2 10 shared papers
NADH-ubiquinone oxidoreductase chain 4 10 shared papers
Cytochrome b 5 shared papers
NADH-ubiquinone oxidoreductase chain 1 8 shared papers
ATP synthase F(0) complex subunit a 8 shared papers
NADH-ubiquinone oxidoreductase chain 3 8 shared papers
ATP synthase F(0) complex subunit 8 7 shared papers
NADH-ubiquinone oxidoreductase chain 4L 7 shared papers
Iron-sulfur cluster transfer protein NUBPL 1 shared papers
NADH-ubiquinone oxidoreductase chain 6 6 shared papers
Cytochrome c oxidase subunit 3 3 shared papers
Cytochrome c oxidase subunit 1 6 shared papers

Literature · 25 cited papers

N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency. Nat. Genet. · 2010
Mutations in the ND5 subunit of complex I of the mitochondrial DNA are a frequent cause of oxidative phosphorylation disease. J. Med. Genet. · 2007
The role of the ND5 gene in LHON: characterization of a new, heteroplasmic LHON mutation. Ann. Neurol. · 2005
Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF. Arch. Neurol. · 2005
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
A missense mutation in the mitochondrial ND5 gene associated with a Leigh-MELAS overlap syndrome. Neurology · 2003
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J. Biol. Chem. · 2003
Is the mitochondrial complex I ND5 gene a hot-spot for MELAS causing mutations? Ann. Neurol. · 2003
… and 13 more in the literature graph

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