Immunogen
Purified recombinant human PEPCK (C-terminus) protein fragments expressed in E.coli.
Specificity
PEPCK Monoclonal Antibody detects endogenous levels of PEPCK protein.
Formulation
Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.
Dilution rate
Western Blot: 1/1000 - 1/2000. Not yet tested in other applications.
Purification process (Immunogen)
Affinity purification
Background
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],
Function
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
Protein name
Phosphoenolpyruvate carboxykinase [GTP] mitochondrial
Other name
PCK2; PEPCK2; Phosphoenolpyruvate carboxykinase [GTP]; mitochondrial; PEPCK-M; Phosphoenolpyruvate carboxylase
Fields
>>Glycolysis / Gluconeogenesis;>>Citrate cycle (TCA cycle);>>Pyruvate metabolism;>>Metabolic pathways;>>PPAR signaling pathway;>>FoxO signaling pathway;>>PI3K-Akt signaling pathway;>>AMPK signaling pathway;>>Insulin signaling pathway;>>Adipocytokine signaling pathway;>>Glucagon signaling pathway;>>Insulin resistance;>>Proximal tubule bicarbonate reclamation
Human protein sequence Database
Q16822
Mouse protein sequence database
Q8BH04
Rat protein sequence database
Cellular localization
Mitochondrion.
Tissue expression
Liver,Neuroblastoma,Placenta,
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