Human Ketohexokinase Alexa Fluor® 350-conjugated Antibody

Applications

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Preparation and Storage

Background: Ketohexokinase

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).

Product Datasheets

Related Research Areas

• Glycobiology-related Enzymes
• Intracellular Kinases
• Search Glycobiology-related Enzymes
• Search Kinases and Phosphatases