Large neutral amino acid supplementation as an alternative to the phenylalanine-restricted diet in adults with phenylketonuria: evidence from adult Pah-enu2 mice
Author:
Funder
National PKU Alliance
Stofwisselkracht
Publisher
Elsevier BV
Subject
Clinical Biochemistry,Nutrition and Dietetics,Molecular Biology,Biochemistry,Endocrinology, Diabetes and Metabolism
Reference37 articles.
1. Phenylketonuria;Blau;Lancet,2010
2. The neurochemistry of phenylketonuria;Surtees;Eur J Pediatr,2000
3. Brain dysfunction in phenylketonuria: is phenylalanine toxicity the only possible cause?;van Spronsen;J Inherit Metab Dis,2009
4. Pathogenesis of cognitive dysfunction in phenylketonuria: review of hypotheses;de Groot;Mol Genet Metab,2010
5. Large neutral amino acid supplementation exerts its effect through three synergistic mechanisms: proof of principle in phenylketonuria mice;van Vliet;PLoS One,2015
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