{"product_id":"abcam-ab89784","title":"Abcam, ab89784, Anti-PCCA antibody","description":"\u003cp\u003eSize: 50µg\u003cbr\u003e\nMouse Polyclonal PCCA antibody. Carrier free. Suitable for WB and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human PCCA.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Mouse,\u003cbr\u003e\nClonality:Polyclonal,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:Yes,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Recombinant Full Length Protein corresponding to Human PCCA.P05165\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, 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\nPCCA also known as propionyl-CoA carboxylase alpha chain is an essential enzyme component in humans. It has a molecular mass of approximately 81.4 kDa and is primarily expressed in the liver and kidney. PCCA forms a biotin-dependent enzyme that catalyzes the carboxylation of propionyl-CoA to form D-methylmalonyl-CoA. This action is an important step in the mitochondrial beta-oxidation of odd-chain fatty acids and some amino acids.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nPCCA is a part of the propionyl-CoA carboxylase complex which functions as a heterododecameric complex involving both alpha and beta subunits. This enzyme plays an important role in energy metabolism facilitating the conversion of propionyl-CoA into more useful forms for cellular processes. The proper functioning of this complex ensures the breakdown and assimilation of important metabolites aiding in the elimination of toxic intermediates produced during the metabolism of branched-chain amino acids as well as odd-chain fatty acids.\u003cbr\u003e\nPathways\u003cbr\u003e\nThe catabolism facilitated by PCCA involves the metabolic pathways of branched-chain amino acids and odd-numbered fatty acids. It fits within the larger methylmalonyl-CoA mutase pathway where it transforms its substrate into an immediate precursor for succinyl-CoA production. The activity in this pathway links PCCA to other proteins such as methylmalonyl-CoA mutase which subsequently acts to convert methylmalonyl-CoA to succinyl-CoA a critical step for entry into the tricarboxylic acid (TCA) cycle.\u003cbr\u003e\nPCCA defects are linked to propionic acidemia a serious metabolic disorder characterized by the accumulation of propionic acid. This disease results from genetic mutations affecting the normal function of the PCCA protein leading to toxic buildup of metabolites in blood and tissues. The disorder can cause severe metabolic acidosis and can impact neurological development. Additionally PCCA interacts with various cellular proteins such as methylmalonyl-CoA mutase which if disrupted also associates with metabolic disorders exemplifying the interdependent relationship between components in metabolic pathways.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46852273275049,"sku":"ab89784","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab89784","provider":"Iright","version":"1.0","type":"link"}