Future role of large neutral amino acids in transport of phenylalanine into the brain
R Matalon, S Surendran, KM Matalon, S Tyring… - …, 2003 - publications.aap.org
Objective. The treatment of phenylketonuria (PKU) in children and adults has been difficult
because of erosion of dietary adherence, leading to poor school performance, impairment of
executive functioning, loss of IQ, and deterioration of white matter in the brain. Mutant PKU
mice produced by exposure to N-ethyl-N′-nitrosourea (ENU) were used to examine the
effect of large neutral amino acid (LNAA) supplementation on brain and blood
phenylalanine (Phe). Methods. Mice with PKU, genotype ENU 2/2 with features of classical …
because of erosion of dietary adherence, leading to poor school performance, impairment of
executive functioning, loss of IQ, and deterioration of white matter in the brain. Mutant PKU
mice produced by exposure to N-ethyl-N′-nitrosourea (ENU) were used to examine the
effect of large neutral amino acid (LNAA) supplementation on brain and blood
phenylalanine (Phe). Methods. Mice with PKU, genotype ENU 2/2 with features of classical …
