|Application ||IHC-P, FC, WB, E|
|Other Accession||P13803, Q99LC5, Q8HXY0, Q2KJE4, NP_000117.1|
|Predicted||Bovine, Monkey, Rat|
|Calculated MW||35080 Da|
|Other Names||Electron transfer flavoprotein subunit alpha, mitochondrial, Alpha-ETF, ETFA|
|Target/Specificity||This ETFA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 276-304 amino acids from the C-terminal region of human ETFA.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||ETFA Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase (PubMed:27499296, PubMed:15159392, PubMed:15975918, PubMed:9334218, PubMed:10356313). It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase) (PubMed:9334218). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism (PubMed:12815589, PubMed:1882842, PubMed:1430199).|
|Cellular Location||Mitochondrion matrix.|
Provided below are standard protocols that you may find useful for product applications.
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.
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)