{"product_id":"abcam-ab110302","title":"Abcam, ab110302, Anti-HADHA + HADHB antibody [4A8BG12]","description":"\u003cp\u003eSize: 100µg\u003cbr\u003e\nMouse Monoclonal HADHA antibody. Suitable for IHC-P, ICC, IP, Flow Cyt and reacts with Human samples. Cited in 3 publications.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Mouse,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:4A8BG12,\u003cbr\u003e\nIsotype:IgG1,\u003cbr\u003e\nLight chain type:kappa,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:IP, ICC, IHC-P, Flow CytSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nWant a custom formulation?\u003cbr\u003e\nThis antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Immunogen, Purification notes-ab110302 was produced in vitro using hybridomas grown in serum-free medium, and then purified by affinity purification., Storage buffer-pH: 7.5 Preservative: 0.02% Sodium azide Constituents: HEPES buffered saline, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Storage information-Do Not Freeze\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nHADHA (Hydroxyacyl-CoA Dehydrogenase Trifunctional Multienzyme Complex Subunit Alpha) and HADHB (Trifunctional Protein Subunit Beta) function mechanically as essential components of the mitochondrial trifunctional protein (MTP). These proteins facilitate the three-step process of fatty acid beta-oxidation in the mitochondria. HADHA and HADHB work in tandem to carry out dehydrogenase hydratase and thiolase activities. HADHA has a molecular mass of about 82 kDa while HADHB is approximately 51 kDa. These proteins express primarily in tissues with high energy demands including heart liver and skeletal muscle.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nFatty acid breakdown plays an indispensable role in cellular energy supply. HADHA and HADHB form an integral part of the MTP complex essential for the normal beta-oxidation of long-chain fatty acids. The complex catalyzes important reactions needed to convert long-chain fatty acids to acetyl-CoA which then enters the TCA cycle for ATP production. An efficient functioning MTP complex ensures cells maintain energy homeostasis particularly when glucose is scarce.\u003cbr\u003e\nPathways\u003cbr\u003e\nLong-chain fatty acid metabolism and energy production rely heavily on HADHA and HADHB within the mitochondrial beta-oxidation pathway. These proteins closely interact with enzymes such as carnitine palmitoyltransferase I (CPT1) and long-chain-fatty-acid—CoA ligase. HADHA and HADHB help in the removal of two-carbon units from fatty acids demonstrating their link to both the lipid metabolism pathway and the TCA cycle forming a bridge to energy conversion processes necessary for ATP synthesis.\u003cbr\u003e\nDefects in HADHA and HADHB relate to inherited metabolic diseases like trifunctional protein deficiency and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD). Impaired activity of these proteins results in an accumulation of unused long-chain fatty acids causing severe energy deficiency and rhabdomyolysis. The malfunctioning of closely related proteins like medium-chain acyl-CoA dehydrogenase (MCAD) could exacerbate energy metabolism disorders highlighting their central role in maintaining metabolic health.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46860550635689,"sku":"ab110302","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab110302","provider":"Iright","version":"1.0","type":"link"}