Ketohexokinase Antibody (1020613) [Alexa Fluor® 532]

• Reactivity: Hu
• Applications: WB, IHC
• Clone: 1020613
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Alexa Fluor 532

Ketohexokinase Antibody (1020613) [Alexa Fluor® 532] Summary

• Immunogen: E. coli-derived human Ketohexokinase
  Met1-Val298
  Accession # P50053
• Specificity: Detects human Ketohexokinase in direct ELISAs.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Purity Statement: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Ketohexokinase Antibody (1020613) [Alexa Fluor® 532]

• EC 2.7.1.3
• Fructokinase
• Hepatic fructokinase
• ketohexokinase (fructokinase)
• Ketohexokinase
• KHK

Background

KHK1(Ketohexokinase) catalyzes conversion of fructose to fructose-1-phosphate (1). It is the first enzyme that catabolizes dietary fructose. Mutation of this protein is the molecular basis for essential fructosuria, a clinically benign condition characterized by the incomplete metabolism of fructose in the liver, leading to its excretion in urine (2, 3). Essential fructosuria does not have any clinical manifestations and no treatment is required. However, deficiency of aldolase B, the second enzyme involved in the metabolism of fructose results in the accumulation of fructose-1-phosphate in the blood, which causes fructosemia or hereditary fructose intolerance (4). High level of fructose-1-phosphate inhibits the production of glucose and results in diminished regeneration of adenosine triphosphate. Patients with fuctosemia have symptoms of elevated uric acid, growth abnormalities, and coma if untreated. Therefore, inhibition of KHK1 may lead to a cure for fructosemia. High level of expression of KHK1 is found in liver, kidney, gut, spleen and pancreas. Low levels of expression of KHK1 is found in heart, muscle, brain, and eye (3). The enzymatic activity of recombinant human KHK1 is measured using a phosphatase-coupled method (5).

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.