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...of the body to metabolize the amino acid phenylalanine. Phenylalanine is normally converted in the human body to tyrosine, another amino acid, by a specific organic catalyst, or enzyme, called phenylalanine hydroxylase. This enzyme is not active in individuals who have phenylketonuria.
Substitutions: As Phenylalanine is an aromatic, hydrophobic, amino acid, it prefers substitution with other amino acids of the same type (see above). It particularly prefers to exchange with Tyrosine, which differs only in that it contains a hydroxyl group in place of the ortho hyddrogen on the benzene ring.