Product Information
Molecular Weight69195Da
Protein NamePhosphoenolpyruvate carboxykinase cytosolic [GTP]
Human Gene Id5105
Human Swiss Prot NoP35558
Mouse Gene Id18534
Mouse Swiss Prot NoQ9Z2V4
Rat Gene Id362282
Rat Swiss Prot NoP07379
SpecificityPEPCK-C Polyclonal Antibody detects endogenous levels of PEPCK-C protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NamePCK1; PEPCK1; Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; PEPCK-C; Phosphoenolpyruvate carboxylase
Observed Band(KD)65
Functioncatalytic activity:GTP + oxaloacetate = GDP + phosphoenolpyruvate + CO(2).,cofactor:Binds 1 manganese ion per subunit.,disease:Defects in PCK1 are the cause of cytosolic phosphoenolpyruvate carboxykinase deficiency (cytosolic PEPCK deficiency) [MIM:261680]. 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.,enzyme regulation:Activity is affected by a number of hormones regulating this metabolic process (such as glucagon, insulin, or glucocorticoids).,function:Catalyzes the conversion of oxaloacetate (OAA) to phosphoenolpyruvate (PEP), the rate-limiti
Subcellular LocationCytoplasm, cytosol . Endoplasmic reticulum . Phosphorylation at Ser-90 promotes translocation to the endoplasmic reticulum. .
ExpressionMajor sites of expression are liver, kidney and adipocytes.
Backgroundphosphoenolpyruvate carboxykinase 1(PCK1) Homo sapiens This gene is a main control point for the regulation of gluconeogenesis. The cytosolic enzyme encoded by this gene, along with GTP, catalyzes the formation of phosphoenolpyruvate from oxaloacetate, with the release of carbon dioxide and GDP. The expression of this gene can be regulated by insulin, glucocorticoids, glucagon, cAMP, and diet. Defects in this gene are a cause of cytosolic phosphoenolpyruvate carboxykinase deficiency. A mitochondrial isozyme of the encoded protein also has been characterized. [provided by RefSeq, Jul 2008],