{"product_id":"abcam-ab54477","title":"Abcam, ab54477, Anti-HADHA antibody","description":"\u003cp\u003eSize: 50µg\u003cbr\u003e\nRabbit Polyclonal HADHA antibody. Suitable for WB and reacts with Human, Mouse, Rat samples. Cited in 51 publications. Immunogen corresponding to Synthetic Peptide within Human HADHA.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Polyclonal,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Mouse, Rat, Human,\u003cbr\u003e\nApplications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Synthetic Peptide within Human HADHA. The exact immunogen used to generate this antibody is proprietary information.P40939\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nThis product is manufactured by BioVision, an Abcam company and was previously called 3721 TFP1\/HADHA Antibody.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-Preservative: 0.01% Thimerosal (merthiolate)Constituents: PBS, 30% Glycerol (glycerin, glycerine), 0.5% BSA, Shipped at conditions-Blue Ice, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nThe HADHA protein also called hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha is an essential component of the mitochondrial trifunctional protein complex. This protein has a molecular mass of about 79 kDa and is expressed mainly in tissues with high fatty acid oxidation rates like liver heart and muscle. HADHA plays a significant role in the beta-oxidation of long-chain fatty acids acting on hydroxyacyl-CoA substrates during this critical metabolic process.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nHADHA is a part of the mitochondrial trifunctional protein complex which consists of four alpha and four beta subunits. It facilitates the hydration of enoyl-CoA to 3-hydroxyacyl-CoA and the subsequent dehydrogenation to 3-ketoacyl-CoA. This enzyme works closely with its partner the HADHB protein to carry out these reactions efficiently. These functions are important for energy production as they are steps in the breakdown of fatty acids necessary for ATP generation.\u003cbr\u003e\nPathways\u003cbr\u003e\nHADHA participates in the mitochondrial beta-oxidation pathway an essential pathway for energy production from fats. Alongside HADHB it catalyzes key reactions that allow the progressive shortening of fatty acid chains which further feeds into the citric acid cycle. This pathway links HADHA not only to HADHB but also to other enzymes involved in lipid metabolism and energy homeostasis including medium-chain specific acyl-CoA dehydrogenase (MCAD) reflecting its role in comprehensive metabolic networks.\u003cbr\u003e\nDefects in HADHA are associated with mitochondrial trifunctional protein deficiency and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Both disorders disrupt normal fatty acid oxidation leading to a spectrum of symptoms including hypoketotic hypoglycemia and cardiomyopathy. These conditions highlight the relationship between HADHA and other proteins involved in fatty acid metabolism such as HADHB further highlighting their collective role in maintaining cellular energy balance.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46855727743145,"sku":"ab54477","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab54477","provider":"Iright","version":"1.0","type":"link"}