|Application ||FC, WB, 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.|
|Function||Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:29915090, PubMed:30850536). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long- chain fatty acids (PubMed:30850536). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3- ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536).|
|Cellular Location||Mitochondrion. Mitochondrion inner membrane Mitochondrion outer membrane. Endoplasmic reticulum. Note=Protein stability and association with membranes require HADHA|
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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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