ETF-alpha,Electron transfer flavoprotein subunit alpha,electron-transfer-flavoprotein, alpha polypeptide,mitochondrial,Alpha ETF,Alpha-ETF,Electron transfer flavoprotein alpha polypeptide,Electron transfer flavoprotein alpha subunit,Electron transfer flavoprotein subunit alpha,Electron transfer flavoprotein subunit alpha mitochondrial,Electron transfer flavoprotein subunit alpha, mitochondrial,Electron transferring flavoprotein alpha polypeptide,EMA,ETFA,ETFA_HUMAN,GA2.
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. [provided by RefSeq, Jul 2008].