Abstract
Abstract— Phenylalanine levels approaching those found in clinical phenylketonuria were produced in the brain of suckling rats by injections of p‐chlorophenylalanine and L‐phenylalanine. The predominant aromatic acid metabolite found in the brain of these animals was phenylacetic acid with decreasing amounts of phenylpyruvic, phenyllactic, and mandelic acids.The disposition of [3H]pyridoxamine in the brain of normal and hyperphenylalaninemic animals was found to be similar. Pyridoxamine was rapidly phosphorylated in the brain, and excess vitamer was converted mainly to pyridoxal. Pyridoxamine, when injected repeatedly, was effective in significantly reducing the amount of phenylacetate that accumulated in the brain over a period of 6 h. The significance of these findings is discussed.
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