Electron Transfer Flavoprotein Alpha Polypeptide (ETFa) Antibody – Rabbit Polyclonal

ETFA: The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl- CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Defects in ETFA are the cause of glutaric aciduria type 2A (GA2A); also known as glutaricaciduria IIA. GA2A is 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. Belongs to the ETF alpha-subunit/FixB family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 9|9 B

Cellular Component: mitochondrial electron transfer flavoprotein complex; mitochondrion

Molecular Function: electron transfer activity; FAD binding; oxidoreductase activity; protein binding

Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; transport