{"product_id":"abcam-ab112998","title":"Abcam, ab112998, Anti-GCDH\/GCD antibody [3E9BA3BF5]","description":"\u003cp\u003eSize: 100µg\u003cbr\u003e\nMouse Monoclonal GCDH\/GCD antibody. Suitable for Flow Cyt, ICC\/IF and reacts with Human samples. Cited in 1 publication.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Mouse,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:3E9BA3BF5,\u003cbr\u003e\nIsotype:IgG1,\u003cbr\u003e\nLight chain type:kappa,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:Flow Cyt, ICC\/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:The exact immunogen used to generate this antibody is proprietary information.\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-Proprietary technique, Purification notes-ab112998 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation. Monoclonal purity was near homogeneity as judged by SDS-PAGE (\u0026gt;95%)., Storage buffer-pH: 7.5 Preservative: 0.02% Sodium azide Constituents: 99.98% HEPES buffered saline, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nGCDH also known as glutaryl-CoA dehydrogenase is an enzyme involved in the catabolic breakdown of specific amino acids. This protein harbors a mass of approximately 45 kDa and functions primarily within the mitochondria. GCDH expression occurs in various tissues including the liver kidney and brain. The enzyme plays a mechanical role by catalyzing the decarboxylation of glutaryl-CoA to crotonyl-CoA facilitating the removal of carbon dioxide in this biochemical process.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThis enzyme acts as an important component in the lysine hydroxylysine and tryptophan degradation pathways. GCDH does not form part of a larger enzyme complex but performs its function independently ensuring the proper degradation and metabolism of amino acids. Its activity aids in maintaining metabolic homeostasis preventing the accumulation of harmful intermediates within cells. GCDH may interact with cofactors like flavin adenine dinucleotide (FAD) to carry out its enzymatic activity.\u003cbr\u003e\nPathways\u003cbr\u003e\nGCDH plays a significant role in the lysine and tryptophan metabolism pathways. Within these pathways GCDH helps convert glutaryl-CoA a molecule derived from amino acid catabolism into intermediates ultimately leading to energy production. The enzyme's functionality connects with other mitochondrial enzymes like isovaleryl-CoA dehydrogenase (IVD) which also participates in amino acid breakdown. Proper interaction and coordination within these pathways are essential for maintaining metabolic balance in the cell.\u003cbr\u003e\nGCDH is closely linked to glutaric aciduria type 1 (GA1) an inborn error of metabolism. This disorder arises from mutations in the GCDH gene leading to insufficient enzyme activity and subsequent buildup of glutaric acid. This accumulation can cause severe neurological damage over time if untreated. GA1 can show genetic connections with other metabolic disorders involving enzymes like medium-chain acyl-CoA dehydrogenase (MCAD). Understanding these interactions helps in diagnosing and managing such metabolic disorders.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46855352615081,"sku":"ab112998","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab112998","provider":"Iright","version":"1.0","type":"link"}