Recombinant Human Ketohexokinase Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 8177-HK

R&D Systems, part of Bio-Techne
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8177-HK-020
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Key Product Details

Source

E. coli

Accession #

P50053

Conjugate

Unconjugated

Applications

Enzyme Activity

View all Ketohexokinase Proteins and Enzymes »

Product Specifications

Source

E. coli-derived human Ketohexokinase protein
Met1-Val298, with C-terminal 6-His tag

Purity

>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane.

Endotoxin Level

<0.1 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Met1

Predicted Molecular Mass

34 kDa

SDS-PAGE

28-32 kDa, reducing conditions

Activity

Measured by its ability to phosphorylate D-(-)-fructose.
The specific activity is >1,100 pmol/min/μg, as measured under the described conditions.

Formulation, Preparation and Storage

8177-HK
Formulation Supplied as a 0.2 μm filtered solution in Tris, NaCl, Brij-35 and DTT.
Shipping The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 6 months from date of receipt, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: Ketohexokinase

KHK1 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).

References

  1. Trinh, C.H. et al. (2009) Acta. Crystallogr. D Biol Crystallogr. 65:201.
  2. Zhang, X. et al. (2011) Bioorg. Med. Chem. Lett. 21:4762.
  3. Bonthron, D.T. et al. (1994) Hum. Mol. Genet. 3:1627.
  4. Kaiser, U.B. and Hegele, R. A. (1991). Am. J. Med. Sci. 302: 364.
  5. Wu, Z.L. (2011) PLoS One 6:e23172.

Alternate Names

Fructokinase, KHK

Entrez Gene IDs

3795 (Human); 16548 (Mouse); 25659 (Rat)

Gene Symbol

KHK

UniProt

P50053

Additional Ketohexokinase Products

Product Documents for Recombinant Human Ketohexokinase Protein, CF

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Product Specific Notices for Recombinant Human Ketohexokinase Protein, CF

For research use only

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