功能： catalytic 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-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.,miscellaneous:In eukaryotes there are two isozymes: a cytoplasmic one and a mitochondrial one.,pathway:Carbohydrate biosynthesis; gluconeogenesis.,similarity:Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.,subunit:Monomer.,tissue specificity:Major sites of expression are liver, kidney and adipocytes.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm, cytosol . Endoplasmic reticulum . Phosphorylation at Ser-90 promotes translocation to the endoplasmic reticulum. .
组织表达： Major sites of expression are liver, kidney and adipocytes.
功能： catalytic 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-limiting step in the metabolic pathway that produces glucose from lactate and other precursors derived from the citric acid cycle.,miscellaneous:In eukaryotes there are two isozymes: a cytoplasmic one and a mitochondrial one.,pathway:Carbohydrate biosynthesis; gluconeogenesis.,similarity:Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.,subunit:Monomer.,tissue specificity:Major sites of expression are liver, kidney and adipocytes.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Cytoplasm, cytosol . Endoplasmic reticulum . Phosphorylation at Ser-90 promotes translocation to the endoplasmic reticulum. .
组织表达： Major sites of expression are liver, kidney and adipocytes.
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