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GCK Recombinant Mouse mAb

GCK Recombinant Mouse mAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF
Reactivity Human, Mouse, Rat
Host Mouse
Clonality Recombinant
Physical State Liquid
Isotype IgG1, Kappa
Purity affinity purified by Protein G
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the hexokinase family.
SUBUNIT Monomer.
DISEASE Defects in GCK are the cause of maturity-onset diabetes of the young type 2 (MODY2) [MIM:125851]; also shortened MODY-2. MODY is a form of diabetes that is characterized by an autosomal dominant mode of inheritance, onset in childhood or early adulthood (usually before 25 years of age), a primary defect in insulin secretion and frequent insulin-independence at the beginning of the disease. Defects in GCK are the cause of familial hyperinsulinemic hypoglycemia type 3 (HHF3) [MIM:602485]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions Hexokinases phosphorylate glucose to produce glucose 6 phosphate, thus committing glucose to the glycolytic pathway. Alternative splicing of this gene results in three tissue specific forms of glucokinase, one found in pancreatic islet beta cells and two found in liver. The protein localizes to the outer membrane of mitochondria. In contrast to other forms of hexokinase, this enzyme is not inhibited by its product glucose 6 phosphate but remains active while glucose is abundant. Mutations in this gene have been associated with non insulin dependent diabetes mellitus, also called maturity onset diabetes of the young, type 2; mutations have also been associated with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).
Additional Information
Target/Specificity Isoform 1 is expressed in pancreas. Isoform 2 and isoform 3 is expressed in liver.
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:50-200,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Research Areas

BACKGROUND

Hexokinases phosphorylate glucose to produce glucose 6 phosphate, thus committing glucose to the glycolytic pathway. Alternative splicing of this gene results in three tissue specific forms of glucokinase, one found in pancreatic islet beta cells and two found in liver. The protein localizes to the outer membrane of mitochondria. In contrast to other forms of hexokinase, this enzyme is not inhibited by its product glucose 6 phosphate but remains active while glucose is abundant. Mutations in this gene have been associated with non insulin dependent diabetes mellitus, also called maturity onset diabetes of the young, type 2; mutations have also been associated with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).

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