|Application ||WB, FC, E|
|Other Accession||Q8HXX4, O46629|
|Calculated MW||51294 Da|
|Antigen Region||315-342 aa|
|Other Names||Trifunctional enzyme subunit beta, mitochondrial, TP-beta, 3-ketoacyl-CoA thiolase, Acetyl-CoA acyltransferase, Beta-ketothiolase, HADHB|
|Target/Specificity||This HADHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 315-342 amino acids from the C-terminal region of human HADHB.|
|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||HADHB Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Cellular Location||Mitochondrion. Mitochondrion inner membrane Mitochondrion outer membrane Endoplasmic reticulum|
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Provided below are standard protocols that you may find useful for product applications.
HADHB encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.
Purevsuren, J., et al. Mol. Genet. Metab. 98(4):372-377(2009)
Bogenhagen, D.F., et al. J. Biol. Chem. 283(6):3665-3675(2008)
Wang, R., et al. Zhonghua Fu Chan Ke Za Zhi 41(10):672-675(2006)
Hillier, L.W., et al. Nature 434(7034):724-731(2005)
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