Question

Individuals with the genetic disease phenylketonuria (PKU) lack a single enzyme called phenylalanine hydroxylase, which converts the amino acid phenylalanine to the amino acid tyrosine. If this conversion does not happen, phenylalanine builds up and becomes toxic to the central nervous system, causing serious problems for the individual. The management of this disease is to avoid phenylalanine by severely limiting the amount of protein in the diet. How would this help?

Answer

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