SKU: WM0518 Categories: ,

PEPCK Monoclonal Antibody

Price range: $268.00 through $328.00

SKU: WM0518-50
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Product details

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],

Specifications

Size50μL/100μL
TargetPCK2
ReactivityHuman, Mouse, Rat, Bovine, Dog, Pig
ApplicationsWB
MW(Calculated)71kD
MW(Observed)
Host SpeciesMouse
Isotype
Conjugate/ModificationUnmodified
Recommended Dilution RatioWB 1:1000-1:2000; Not yet tested in other applications.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
PurificationAffinity purification
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone Number5G12
ImmunogenPurified recombinant human PEPCK (C-terminus) protein fragments expressed in E.coli.
SpecificityPEPCK Monoclonal Antibody detects endogenous levels of PEPCK protein.
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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