NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8
Also known as: NDUFA8
Function
Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Classification
- Family (Pfam)
- PF06747 CHCH
- InterPro
- CHCH, NDUFA8
- Functional cluster
- Nucleotide & Amino-Acid Biosynthesis Enzymes
Experimental structures · PDB · 7
A predicted model is available from AlphaFold.
Gene Ontology · 9
- GO:0005743 mitochondrial inner membrane
- GO:0005758 mitochondrial intermembrane space
- GO:0005739 mitochondrion
- GO:0045271 respiratory chain complex I
- GO:0008137 NADH dehydrogenase (ubiquinone) activity
- GO:0044877 protein-containing complex binding
- GO:0009060 aerobic respiration
- GO:0006120 mitochondrial electron transport, NADH to ubiquinone
- GO:0042776 proton motive force-driven mitochondrial ATP synthesis
Disease associations
- mitochondrial complex I deficiency, nuclear type 37 MONDO:0030997
Drugs targeting this protein · 2
- METFORMIN HYDROCHLORIDE inhibitor
- ME-344 inhibitor
Related proteins · sequence + function similarity
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 1.00
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 0.99
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 0.98
- NADH-ubiquinone oxidoreductase 20.8 kDa subunit 0.74
- Cytochrome c oxidase assembly protein COX19 0.71
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 0.70
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8-B 0.69
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 0.68
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 0.68
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 0.68
Co-cited proteins · studied together in the literature
- Cytochrome b-c1 complex subunit Rieske, mitochondrial 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 2 shared papers
- NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 2 shared papers
- Cytochrome c oxidase assembly protein COX19 1 shared papers
- Cytochrome c oxidase assembly factor 4 homolog, mitochondrial 1 shared papers
- FAD-linked sulfhydryl oxidase ALR 1 shared papers
- COX assembly mitochondrial protein homolog 1 shared papers
- Cytochrome c oxidase assembly protein COX19 1 shared papers
- Mitochondrial intermembrane space import and assembly protein 40 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 1 shared papers
- NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 1 shared papers
- Distal membrane-arm assembly complex protein 1 1 shared papers
Literature · 15 cited papers
- Biallelic mutations in NDUFA8 cause complex I deficiency in two siblings with favorable clinical evolution. Mol. Genet. Metab. · 2020
- A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency. Clin. Genet. · 2020
- Accessory subunits are integral for assembly and function of human mitochondrial complex I. Nature · 2016
- 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
- Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells. Mol. Biol. Cell · 2013
- NDUFB7 and NDUFA8 are located at the intermembrane surface of complex I. FEBS Lett. · 2011
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- The consensus coding sequences of human breast and colorectal cancers. Science · 2006
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- DNA sequence and analysis of human chromosome 9. Nature · 2004
- Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
- … and 3 more in the literature graph
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