Animal models of brain dysfunction in phenylketonuria
AE Martynyuk, FJ Van Spronsen… - Molecular genetics and …, 2010 - Elsevier
Phenylketonuria (PKU) is a metabolic disorder that results in significant brain dysfunction if
untreated. Although phenylalanine restricted diets instituted at birth have clearly improved
PKU outcomes, neuropsychological deficits and neurological changes still represent
substantial problems. The specific mechanisms by which Phe affects the brains of
individuals with PKU are yet fully determined. The use of animal models in PKU research
significantly broadens the possibilities for investigating these mechanisms. This report …
untreated. Although phenylalanine restricted diets instituted at birth have clearly improved
PKU outcomes, neuropsychological deficits and neurological changes still represent
substantial problems. The specific mechanisms by which Phe affects the brains of
individuals with PKU are yet fully determined. The use of animal models in PKU research
significantly broadens the possibilities for investigating these mechanisms. This report …