Phenylketonuria
The ‘building blocks’ of protein are called amino acids. In PKU, due to the lack of an enzyme, the body cannot properly deal with one of the amino acids, phenylalanine. People with PKU therefore need to severely restrict the amount of protein they eat.
A key element in the management of PKU is the use of protein substitutes. Protein substitutes for PKU provide all the amino acids found in protein but no phenylalanine. In combination with the small allowance of regular dietary protein, protein substitutes ensure that an individual with PKU has an adequate intake of protein.