Ketohexokinase is an enzyme that catalyzes the phosphorylation of fructose to produce fructose-1- phosphate, leading to consumption of ATP, formation of AMP. This protein initiates first step in the metabolism of dietary fructose and is an important regulator of hepatic glucose metabolism. It is highly found in liver, renal cortex, and small intestine. Its deficiency causes the benign hereditary metabolic disorder essential fructosuria, leading to fructose being excreted in the urine