{"product_id":"pcb-rabbit-polyclonal-antibody-es6488","title":"PCB Rabbit Polyclonal Antibody - ES6488","description":"\u003cp\u003ePCB Rabbit Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES6488-50, ES6488-100\u003c\/p\u003e\n\n\u003cp\u003eLead times: 1-2 weeks, if manufacturer has product in stock\u003c\/p\u003e\n\n\u003cp\u003eManufacturer\/Ship Location: China\u003c\/p\u003e\n\n\u003cp\u003eBackground: This gene encodes pyruvate carboxylase, which requires biotin and ATP to catalyse the carboxylation of pyruvate to oxaloacetate. The active enzyme is a homotetramer arranged in a tetrahedron which is located exclusively in the mitochondrial matrix. Pyruvate carboxylase is involved in gluconeogenesis, lipogenesis, insulin secretion and synthesis of the neurotransmitter glutamate. Mutations in this gene have been associated with pyruvate carboxylase deficiency. Alternatively spliced transcript variants with different 5' UTRs, but encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008],\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB, IHC, IF, ELISA\u003c\/p\u003e\n\n\u003cp\u003eAlternate Names: PC, Pyruvate carboxylase, mitochondrial, Pyruvic carboxylase, PCB\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: The antiserum was produced against synthesized peptide derived from human PC. AA range:357-406\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eTarget: PCB\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: PCB Polyclonal Antibody detects endogenous levels of PCB protein.\u003c\/p\u003e\n\n\u003cp\u003eDilution: Western Blot: 1\/500 - 1\/2000. IHC-p: 1:100-300 ELISA: 1\/20000. IF 1:100-300 Not yet tested in other applications.\u003c\/p\u003e\n\n\u003cp\u003ePurification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml,\u003c\/p\u003e\n\n\u003cp\u003eFormlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.\u003c\/p\u003e\n\n\u003cp\u003eExpression: Epithelium, Kidney, Liver, Lung,\u003c\/p\u003e \n\n\u003cp\u003eObserved Band (KD): 120kD\u003c\/p\u003e\n\n\u003cp\u003eFields: Citrate cycle (TCA cycle), Pyruvate metabolism, Metabolic pathways, Carbon metabolism, Biosynthesis of amino acids\u003c\/p\u003e\n\n\u003cp\u003eFunction: Catalytic Activity: ATP + pyruvate + HCO(3)(-) = ADP + phosphate + oxaloacetate\u003cbr\u003e\nCofactor: Binds 1 manganese ion per subunit\u003cbr\u003e\nCofactor: Biotin\u003cbr\u003e\nDisease: Defects in PC are the cause of pyruvate carboxylase deficiency (PC deficiency) [MIM: 266150]. PC deficiency leads to lactic acidosis, mental retardation and death. It occurs in three forms: mild or type A, severe neonatal or type B, and a very mild lacticacidemia\u003cbr\u003e\nFunction: Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate\u003cbr\u003e\nOnline Information: Pyruvate carboxylase entry,Pathway: Carbohydrate biosynthesis, gluconeogenesis\u003cbr\u003e\nSimilarity: Conta\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Mitochondrion matrix\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eGene Name: PC\u003c\/p\u003e\n\n\u003cp\u003eProtein Name: Pyruvate carboxylase mitochondrial\u003c\/p\u003e\n\n\u003cp\u003eMouse SwissProt Number: Q05920\u003c\/p\u003e\n\n\u003cp\u003eRat Gene ID: 25104\u003c\/p\u003e\n\n\u003cp\u003eRat SwissProt Number: P52873\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":46313608282291,"sku":"ES6488-50","price":186.0,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":46313608315059,"sku":"ES6488-100","price":372.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/elk_antibodies_d75295e2-ffc1-4e0f-a3e7-87715c8983a9.jpg?v=1793190507","url":"https:\/\/afsbio.com\/products\/pcb-rabbit-polyclonal-antibody-es6488","provider":"AFSBio Inc.","version":"1.0","type":"link"}