Future role of large neutral amino acids in transport of XXXX into the brain.

@entity1 @entity330 @entity19 @entity535 @entity814 @entity331 @entity9901 @entity815
OBJECTIVE: The treatment of @entity814 ( @entity814 ) in @entity1 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 @entity814 @entity19 produced by exposure to @entity330 ( @entity331 ) were used to examine the effect of large neutral @entity535 (LNAA) supplementation on brain and blood @entity815 ( @entity815 ). METHODS: @entity19 with @entity814 , genotype ENU 2/2 with features of classical @entity814 , were supplemented with LNAA while on a normal diet. Two dosages of LNAA were given 0.5 g/kg and 1.0 g/kg by gavage. Blood @entity815 was determined in the experimental, control, and sham-treated @entity19 . Brain @entity815 was determined by magnetic resonance spectroscopy after @entity9901 extraction. Branched-chain @entity535 transferase (BCAT) was determined in brain as a marker for energy metabolism. RESULTS: Blood @entity815 was reduced in the LNAA-treated @entity19 by an average of 15% (0.5 g/kg) and 50% (1.0 g/kg) in 48 hours. There was a sustained decrease in the blood @entity815 levels over a 6-week trial. The untreated @entity19 and sham-treated @entity19 maintained high blood @entity815 throughout the experiments. Brain @entity815 level determined by magnetic resonance spectroscopy showed a decline of 46% after the LNAA treatment. BCAT levels were lower (33%) in the ENU 2/2 @entity19 compared with wild-type. The BCAT normalized in @entity19 with @entity814 that were treated with LNAA. CONCLUSION: The results suggest that giving LNAA lowered brain and blood @entity815 levels in @entity19 with @entity814 . Energy metabolism generated from BCAT also improved in @entity19 with @entity814 after treatment with LNAA. Data from the @entity19 suggest that LNAA should be considered among the strategies to treat @entity814 in @entity1 .
