Where is phenylalanine involved?
Phenylalanine accumulation in the body causes phenylketonuria (PKU). In this case the phenylalanine is not converted into tyrosine due to absence or deficiency of phenylalanine hydroxylase, which attaches OH-group on the phenyl group of phenylalanine to convert it into tyrosine. Symptoms of phenylketonuria include mental retardation and demyelination. Normally newborn babies are tested for phenylketonuria so that giving a low phenyalanine diet can prevent the onset of the disease.