{"product_id":"cst-50257s","title":"CST,  50257S, HADHA (F9M4C) Rabbit Monoclonal Antibody","description":"Monoclonal Antibody for studying HADHA. Validated for Western Blotting,Simple Western™. Available in 2 sizes. Highly specific and rigorously validated in-house, HADHA (F9M4C) Rabbit Monoclonal Antibody (CST #50257) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\nSimple Western™: 1:50 - 1:250\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nHADHA (F9M4C) Rabbit Monoclonal Antibody recognizes endogenous levels of total HADHA protein. This antibody does not cross-react with HADHB protein.\nSpecies Reactivity: Human\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val168 of human HADHA protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nMitochondrial trifunctional enzyme, also known as mitochondrial trifunctional protein (MTP), catalyzes three reactions in the fatty acid β-oxidation pathway. It has two different types of subunits: α\/HADHA and β\/HADHB. HADHA possesses the 2,3-enoyl-CoA hydratase (ECH) and 3-hydroxyacyl-CoA dehydrogenase (HACD) activities, while HADHB has 3-ketoacyl-CoA thiolase activity (1-3). Studies show that HADHA stimulates ketone body production through fatty acid β-oxidation. β-hydroxybutyrate, one of the ketone bodies, inhibits gluconeogenesis (1). Furthermore, studies have identified mutations in that cause MTP deficiency, an autosomal recessive disorder severely restricting long-chain fatty acid oxidation (4-6).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\n3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; ECHA; gastrin-binding protein; GBP; HADH; HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha; hydroxyacyl-CoA dehydrogenase\/3-ketoacyl-CoA thiolase\/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase\/3-ketoacyl-Coenzyme A thiolase\/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; LCEH; LCHAD; Long chain 3-hydroxyacyl-CoA dehydrogenase; long-chain 2-enoyl-CoA hydratase; Long-chain enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; MGC1728; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; Monolysocardiolipin acyltransferase; MTPA; TP-alpha; Trifunctional enzyme subunit alpha, mitochondrial\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H\nSENSITIVITY: Endogenous\nMW (kDa): 80\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46799754821801,"sku":"50257S","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-50257s","provider":"Iright","version":"1.0","type":"link"}