|Other Names||Electron transfer flavoprotein subunit alpha, mitochondrial, Alpha-ETF, ETFA|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||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).|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
ETFA participates in catalyzing the initial step of themitochondrial fatty acid beta-oxidation. It shuttles electronsbetween primary flavoprotein dehydrogenases and the membrane-boundelectron transfer flavoprotein ubiquinone oxidoreductase. Defectsin electron-transfer-flavoprotein have been implicated in type IIglutaricaciduria in which multiple acyl-CoA dehydrogenasedeficiencies result in large excretion of glutaric, lactic,ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovalericacids. Two transcript variants encoding different isoforms havebeen found for this gene.
Ohkuma, A., et al. Muscle Nerve 39(3):333-342(2009)Chiong, M.A., et al. Mol. Genet. Metab. 92 (1-2), 109-114 (2007) :Olsen, J.V., et al. Cell 127(3):635-648(2006)Olsen, J.V., et al. Cell 127(3):635-648(2006)Schiff, M., et al. Mol. Genet. Metab. 88(2):153-158(2006)
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