Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pahenu2 mice
EA Sawin, SG Murali, DM Ney - Molecular genetics and metabolism, 2014 - Elsevier
Phenylketonuria (PKU) is an inborn error of metabolism caused by a deficiency of the
enzyme phenylalanine hydroxylase, which metabolizes phenylalanine (phe) to tyrosine. A
low-phe diet plus amino acid (AA) formula is necessary to prevent cognitive impairment;
glycomacropeptide (GMP) contains minimal phe and provides a palatable alternative to the
AA formula. Our objective was to assess neurotransmitter concentrations in the brain and the
behavioral phenotype of PKU mice (Pah enu2 on the C57Bl/6 background) and how this is …
enzyme phenylalanine hydroxylase, which metabolizes phenylalanine (phe) to tyrosine. A
low-phe diet plus amino acid (AA) formula is necessary to prevent cognitive impairment;
glycomacropeptide (GMP) contains minimal phe and provides a palatable alternative to the
AA formula. Our objective was to assess neurotransmitter concentrations in the brain and the
behavioral phenotype of PKU mice (Pah enu2 on the C57Bl/6 background) and how this is …