{"product_id":"yp-ab-03441-53ul","title":"PEPCK Monoclonal Antibody","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eReaction species:\u003c\/b\u003e Human;Mouse;Rat;Bovine;Dog;Pig\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e PCK2\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein name:\u003c\/b\u003e Phosphoenolpyruvate carboxykinase [GTP] mitochondrial\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e Purified recombinant human PEPCK (C-terminus) protein fragments expressed in E.coli.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e PEPCK Monoclonal Antibody detects endogenous levels of PEPCK protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Monoclonal, Mouse\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e Western Blot: 1\/1000 - 1\/2000. Not yet tested in other applications.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e Affinity purification\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eConcentration:\u003c\/b\u003e mg\/ml\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eStorage:\u003c\/b\u003e -20°C\/1 year\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eOther Names:\u003c\/b\u003e PCK2; PEPCK2; Phosphoenolpyruvate carboxykinase [GTP];  mitochondrial; PEPCK-M; Phosphoenolpyruvate carboxylase\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e This gene encodes a mitochondrial enzyme that catalyzes the conversion of oxaloacetate to phosphoenolpyruvate in the presence of guanosine triphosphate (GTP). A cytosolic form of this protein is encoded by a different gene and is the key enzyme of gluconeogenesis in the liver. Alternatively spliced transcript variants have been described. [provided by RefSeq, Apr 2014],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e catalytic activity:GTP + oxaloacetate = GDP + phosphoenolpyruvate + CO(2).,cofactor:Binds 1 manganese ion per subunit.,cofactor:Manganese.,disease:Defects in PCK2 are the cause of mitochondrial phosphoenolpyruvate carboxykinase deficiency (mitochondrial PEPCK deficiency) [MIM:261650]. PEPCK deficiency is a metabolic disorder resulting from impaired gluconeogenesis. It is a rare disease with less than 10 cases reported in the literature. Clinical characteristics include hypotonia, hepatomegaly, failure to thrive, lactic acidosis and hypoglycaemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.,function:Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"53UL","offer_id":50688889159960,"sku":"YP-Ab-03441-53UL","price":0.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/images_2fca2971-e1d8-4225-b515-ffea88332b9d.png?v=1758557015","url":"https:\/\/danabiosci.com\/products\/yp-ab-03441-53ul","provider":"Dana Bioscience","version":"1.0","type":"link"}