Therapeutic brain modulation with targeted large neutral amino acid supplements in the Pah-enu2 phenylketonuria mouse model

Large Neutral Amino Acid Supplementation Exerts Its Effect through Three Synergistic Mechanisms: Proof of Principle in Phenylketonuria Mice

(2015) Danique van Vliet et al.   PLoS One

Pharmacologic inhibition of L-tyrosine degradation ameliorates cerebral dopamine deficiency in murine phenylketonuria (PKU)

(2014) Cary O. Harding et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Neurocognitive Evidence for Revision of Treatment Targets and Guidelines for Phenylketonuria

(2014) Rianne Jahja et al.   JOURNAL OF PEDIATRICS

Behavioral and Neurochemical Characterization of New Mouse Model of Hyperphenylalaninemia

(2013) Tiziana Pascucci et al.   PLoS One

PRELIMINARY SUPPORT FOR THE ORAL ADMINISTRATION OF VALINE, ISOLEUCINE AND LEUCINE FOR PHENYLKETONURIA

(2010) Mary Kay Jordan et al.   DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY

Large neutral amino acids in the treatment of PKU: from theory to practice

(2010) Francjan J. van Spronsen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Phenylketonuria

(2010) Nenad Blau et al.   LANCET

Brain dysfunction in phenylketonuria: Is phenylalanine toxicity the only possible cause?

(2009) F. J. van Spronsen et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Phenylketonuria: High plasma phenylalanine decreases cerebral protein synthesis

(2009) Marieke Hoeksma et al.   MOLECULAR GENETICS AND METABOLISM

Animal models of brain dysfunction in phenylketonuria☆

(2009) A.E. Martynyuk et al.   MOLECULAR GENETICS AND METABOLISM