产品名称
PEPCK-C Rabbit Polyclonal Antibody
别名
PCK1; PEPCK1; Phosphoenolpyruvate carboxykinase, cytosolic [GTP]; PEPCK-C; Phosphoenolpyruvate carboxylase
蛋白名称
Phosphoenolpyruvate carboxykinase cytosolic [GTP]
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=5105
Human Swissprot No.
P35558
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P35558/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18534
Mouse Swissprot No.
Q9Z2V4
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9Z2V4
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=362282
Rat Swissprot Link
http://www.uniprot.org/uniprot/P07379
免疫原
The antiserum was produced against synthesized peptide derived from the Internal region of human PCK1. AA range:491-540
特异性
PEPCK-C Polyclonal Antibody detects endogenous levels of PEPCK-C protein.
稀释度
WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:20000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Major sites of expression are liver, kidney and adipocytes.
细胞定位
Cytoplasm, cytosol . Endoplasmic reticulum . Phosphorylation at Ser-90 promotes translocation to the endoplasmic reticulum. .
信号通路
Glycolysis / Gluconeogenesis;Citrate cycle (TCA cycle);Pyruvate metabolism;PPAR;Insulin_Receptor;Adipocytokine;
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.