Recombinant Human Electron transfer flavoprotein subunit alpha, mitochondrial (ETFA)

Uniprot Id

P13804

Target Species

Human

Target Name

ETFA

Target Full Name

Electron transfer flavoprotein subunit alpha, mitochondrial

Target Function

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

Target Involvement

Glutaric aciduria 2A (GA2A)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

ETF alpha-subunit/FixB family

Target Research Area

others

Target Synonyms

Alpha ETF; Alpha-ETF; Electron transfer flavoprotein alpha polypeptide; Electron transfer flavoprotein alpha subunit; Electron transfer flavoprotein subunit alpha mitochondrial; Electron transfer flavoprotein subunit alpha; mitochondrial; Electron transferring flavoprotein alpha polypeptide; EMA; ETFA; ETFA_HUMAN; GA2; Glutaric aciduria II

Target Background

ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene.