反应种属
Human;Mouse;Rat;Bovine;Dog;Pig
蛋白名称
Phosphoenolpyruvate carboxykinase [GTP] mitochondrial
免疫原
Purified recombinant human PEPCK (C-terminus) protein fragments expressed in E.coli.
特异性
PEPCK Monoclonal Antibody detects endogenous levels of PEPCK protein.
组成
Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.
稀释比例
Western Blot: 1/1000 - 1/2000. Not yet tested in other applications.
纯化工艺
Affinity purification
其他名称
PCK2; PEPCK2; Phosphoenolpyruvate carboxykinase [GTP]; mitochondrial; PEPCK-M; Phosphoenolpyruvate carboxylase
背景
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],
功能
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
