SKU: WR5764 Categories: ,

U-Blot® PEPCK Rabbit mAb

Price range: $268.00 through $328.00

SKU: WR5764-100
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Specifications

Size50μL/100μL
TargetPCK2
ReactivityHuman, Mouse, Rat
ApplicationsWB, IHC, IF, ELISA
MW(Calculated)71kD
MW(Observed)68kD
Host SpeciesRabbit
IsotypeIgG,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioIHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRM1727R
ImmunogenThe specific immunogen used to produce this antibody is proprietary information.
SpecificityEndogenous
Gene NamePCK2
Protein NamePhosphoenolpyruvate carboxykinase [GTP] mitochondrial
Other NamePEPCK; / PCK2; / PEPCK2; / Phosphoenolpyruvate carboxykinase [GTP]; / mitochondrial; / PEPCK-M; / Phosphoenolpyruvate carboxylase
SpeciesHuman
Gene ID5106
UniprotQ16822,
Species.1Mouse
Gene ID.174551
Uniprot.1Q8BH04
Species.2
Gene ID.2
Uniprot.2
Cellular LocalizationMitochondrion.
Tissue ExpressionLiver,Neuroblastoma,Placenta,
Signaling_pathwayOrganismal Systems >> Endocrine system >> Insulin signaling pathway;Organismal Systems >> Endocrine system >> Glucagon signaling pathway;Organismal Systems >> Endocrine system >> Adipocytokine signaling pathway;Organismal Systems >> Endocrine system >> PPAR signaling pathway;Environmental Information Processing >> Signal transduction >> FoxO signaling pathway;Environmental Information Processing >> Signal transduction >> PI3K-Akt signaling pathway;Environmental Information Processing >> Signal transduction >> AMPK signaling pathway
Research Areas>>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
FunctionCatalytic 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.,
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