ITM1074
ITM1074
- Catalog: ITM1074
- Gene/Protein: PCK2
- Product Description: Immunotag™ PEPCK Monoclonal Antibody
504.0000
Price in reward points: 504
| Antibody Specification | |
| Datasheet | 
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| Target Protein | PEPCK |
| Clonality | Monoclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB |
| Recommended Dilution | Western Blot: 1/1000 - 1/2000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat,Bovine,Dog,Pig |
| Host Species | Mouse |
| Immunogen | Purified recombinant human PEPCK (C-terminus) protein fragments expressed in E.coli. |
| Specificity | PEPCK Monoclonal Antibody detects endogenous levels of PEPCK protein. |
| Purification | Affinity purification |
| Form | Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol. |
| Gene Name | PCK2 |
| Accession No. | Q16822 Q8BH04 |
| Alternate Names | PCK2; PEPCK2; Phosphoenolpyruvate carboxykinase [GTP]; mitochondrial; PEPCK-M; Phosphoenolpyruvate carboxylase |
| Description | phosphoenolpyruvate carboxykinase 2, mitochondrial(PCK2) Homo sapiens 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], |
| Cell Pathway/ Category | Glycolysis / Gluconeogenesis,Citrate cycle (TCA cycle),Pyruvate metabolism,PPAR,Insulin_Receptor,Adipocytokine, |
| Protein Expression | Liver,Neuroblastoma,Placenta, |
| Subcellular Localization | mitochondrion,mitochondrial matrix,extracellular exosome, |
| Protein 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 cycle.,miscellaneous:In eukaryotes there are two isozymes: a cytoplasmic one and a mitochondrial one.,pathway:Carbohydrate biosynthesis; gluconeogenesis.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the phosphoenolpyruvate carboxykinase [GTP] family.,subunit:Monomer., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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