Conjugation: FITC: Fluorescein Isothiocyanate
Description of Target: 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.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.
Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human ETFA
Key Reference: Chiong,M.A., Mol. Genet. Metab. 92 (1-2), 109-114 (2007)
Molecular Weight: 35kDa
Peptide Sequence: Synthetic peptide located within the following region:
FRAAAPGQLRRAASLLRFQSTLVIAEHANDSLAPITLNTITAATRLGGEVProduct Format: Liquid. Purified antibody supplied in 1x PBS buffer.
Protein Name: Electron transfer flavoprotein subunit alpha, mitochondrial
Protein Size: 333
Purification: Affinity Purified
Subunit: alpha, mitochondrial
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