NADH-ubiquinone oxidoreductase chain 4L

UniProt P03901 Organism Homo sapiens Gene MT-ND4L, MTND4L, NADH4L, ND4L EC 7.1.1.2

Also known as: MT-ND4L, MTND4L, NADH4L, ND4L

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. Part of the enzyme membrane arm which is embedded in the lipid bilayer and involved in proton translocation.

Classification

Family (Pfam): PF00420 Oxidored_q2
InterPro: NADH_UbQ_OxRdtase_chain4L/K, NUOK/Mnh_C1-like
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 · 8

Disease associations

Leber hereditary optic neuropathy MONDO:0010788

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 4L 1 shared papers
Cytochrome c oxidase subunit 2 8 shared papers
Cytochrome b 4 shared papers
NADH-ubiquinone oxidoreductase chain 3 7 shared papers
NADH-ubiquinone oxidoreductase chain 1 7 shared papers
NADH-ubiquinone oxidoreductase chain 2 7 shared papers
NADH-ubiquinone oxidoreductase chain 5 7 shared papers
NADH-ubiquinone oxidoreductase chain 4 7 shared papers
NADH-ubiquinone oxidoreductase chain 6 6 shared papers
Cytochrome c oxidase subunit 1 7 shared papers
ATP synthase F(0) complex subunit 8 6 shared papers
ATP synthase F(0) complex subunit a 6 shared papers

Literature · 12 cited papers

Architecture of human mitochondrial respiratory megacomplex I2III2IV2. Cell · 2017
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
Mitochondrial genome variation and the origin of modern humans. Nature · 2000
Somatic mutations of the mitochondrial genome in human colorectal tumours. Nat. Genet. · 1998
Comparison between the complete mitochondrial DNA sequences of Homo and the common chimpanzee based on nonchimeric sequences. J. Mol. Evol. · 1996
Phylogenetic analysis of Leber's hereditary optic neuropathy mitochondrial DNA's indicates multiple independent occurrences of the common mutations. Hum. Mutat. · 1995
Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs. Proc. Natl. Acad. Sci. U.S.A. · 1995
Sequence and organization of the human mitochondrial genome. Nature · 1981
Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase. Nature · 1985
Normal variants of human mitochondrial DNA and translation products: the building of a reference data base. Hum. Genet. · 1991

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