|Application ||WB, FC, IHC-P|
|Calculated MW||83000 Da|
|Antigen Region||737-763 aa|
|Other Names||Trifunctional enzyme subunit alpha, mitochondrial, 78 kDa gastrin-binding protein, TP-alpha, Long-chain enoyl-CoA hydratase, Long chain 3-hydroxyacyl-CoA dehydrogenase, HADHA, HADH|
|Target/Specificity||This HADHA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 737-763 amino acids from the C-terminal region of human HADHA.|
|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||HADHA Antibody (C-term) [Knockout Validated] 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:1550553, 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 described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787).|
|Cellular Location||Mitochondrion. Mitochondrion inner membrane Note=Protein stability and association with mitochondrion inner membrane do not require HADHB.|
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Provided below are standard protocols that you may find useful for product applications.
HADHA is the alpha 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 alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities.
Sims,H.F., et.al., Proc. Natl. Acad. Sci. U.S.A. 92 (3), 841-845 (1995)
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