What is PKU?
Phenylketonuria, or PKU is an inborn error in metabolism. It is a defect in the enzyme Phenylalnine hydroxylase, which normally processes the amino acid phenylalanine into tyrosine for use in the body. This defect leads to a build up of phenylalanine in the blood which causes neurological damage. Phenylalanine is found in most dietary protein. For this reason phenylketonurics have to maintain a low protein diet. They supplement their diet with a medical formula that provides protein without phenylalanine, as well as other nutrients. This way people with PKU can feel full and receive all the nutrients they need, despite a limited diet.