Abstract
The urinary excretion of amino acids was examined in phenylketonuric patients on and off low-phenylalanine diets, in hyperphenylalaninemic individuals, and in normal children. In phenylketonuric persons off diets, the pattern of amino acid excretion suggested competitive inhibition of reabsorption of amino acids by the high filtered load of phenylalanine. Hyperphenylalaninemic patients had a generalized aminoaciduria, prompting the hypothesis that they are phenylketonuric individuals who are protected by their increased urinary excretion of phenylalanine.
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