Valine, isoleucine, and leucine: a new treatment for phenylketonuria
HK Berry, RL Brunner, MM Hunt… - American Journal of …, 1990 - jamanetwork.com
• Early treatment of phenylketonuria by dietary phenylalanine restriction prevents brain
damage. Behavioral and cognitive deficits occur when serum phenylalanine levels increase.
Administration of valine, isoleucine, and leucine to patients with phenylketonuria may inhibit
entry of phenylalanine into the brain and reduce its toxic effects on the central nervous
system. Sixteen adolescents and young adults with phenylketonuria participated in double-
blind trials in which a valine, isoleucine, and leucine mixture or a control mixture was given …
damage. Behavioral and cognitive deficits occur when serum phenylalanine levels increase.
Administration of valine, isoleucine, and leucine to patients with phenylketonuria may inhibit
entry of phenylalanine into the brain and reduce its toxic effects on the central nervous
system. Sixteen adolescents and young adults with phenylketonuria participated in double-
blind trials in which a valine, isoleucine, and leucine mixture or a control mixture was given …