Mouse anti-Human/Mouse/Rat sapiens (Human) ETFA Monoclonal Antibody

Function

Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase (PubMed:10356313, PubMed:15159392, PubMed:15975918, PubMed:27499296, PubMed:9334218). It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase) (PubMed:9334218). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism (PubMed:12815589, PubMed:1430199, PubMed:1882842)

Subcellular Location

Mitochondrion matrix

Disease Association

Glutaric aciduria 2A (GA2A) : An autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

Heterodimer composed of ETFA and ETFB (PubMed:15159392, PubMed:15975918, PubMed:8962055, PubMed:9334218). Identified in a complex that contains ETFA, ETFB and ETFRF1 (PubMed:27499296). Interaction with ETFRF1 promotes dissociation of the bound FAD and loss of electron transfer activity (PubMed:27499296). Interacts with MBTPS1; the interaction stabilizes the ETFA/ETFB heterodimer and promotes FAD binding (PubMed:35362222)

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