ETFA Antibody (C-term)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| IHC-P, FC, WB, E |
---|---|
Primary Accession | P13804 |
Other Accession | P13803, Q99LC5, Q8HXY0, Q2KJE4, NP_000117.1 |
Reactivity | Human, Mouse |
Predicted | Bovine, Monkey, Rat |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 35080 Da |
Antigen Region | 276-304 aa |
Gene ID | 2108 |
---|---|
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. |
Dilution | WB~~1:1000 IHC-P~~1:50~100 FC~~1:10~50 |
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. |
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:10356313, PubMed:15159392, PubMed:15975918, PubMed:27499296, PubMed:9334218). 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:1430199, PubMed:1882842). |
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 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.
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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