The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/1000 - 1/10000. Detects a band of approximately 69 kDa (predicted molecular weight: 69 kDa).
应用说明Is unsuitable for IHC-P or IP.
功能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.
组织特异性Major sites of expression are liver, kidney and adipocytes.
通路Carbohydrate biosynthesis; gluconeogenesis.
疾病相关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 hypoglycemia. Autoposy reveals fatty infiltration of both the liver and kidneys. The disorder is transmitted as an autosomal recessive trait.
序列相似性Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.
翻译后修饰Acetylation is increased on addition of glucose and appears to regulate the protein stability.