Oxidative stress in phenylketonuric patients

Abstract

Phenylketonuria is the most frequent disturbance of amino acid metabolism. Untreated patients present mental retardation whose pathophysiology is not completely established. In this work we discuss the oxidative stress in phenylketonuric patients. Several studies have shown reduction in antioxidant defenses, possibly due to dietary restriction of nutrients with antioxidant properties and increase in oxidative damage to biomolecules, probably secondary to increased formation of reactive species. Therefore, antioxidants could be considered an adjuvant therapy in phenylketonuria.