ETFA Antibody (C-term) Blocking peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Primary Accession | P13804 |
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Clone Names | 81201114 |
Gene ID | 2108 |
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Other Names | Electron transfer flavoprotein subunit alpha, mitochondrial, Alpha-ETF, ETFA |
Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
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. |
Name | ETFA |
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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. |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
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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