– Ketohexokinase, also known as Hepatic fructokinase, is a member of the carbohydrate kinase PfkB family. It exits as a homodimer and most abundant in liver, kidney, gut, spleen and pancreas. Low levels also found in adrenal, muscle, brain and eye.This enzyme catalyzes conversion of fructose to fructose-1-phosphate. It is the first enzyme with a specialized pathway that catabolizes dietary fructose. Defects in KHK are the cause of fructosuria.