Abstract
Investigation of a psychomotorically retarded girl showed excretion of abnormal amounts of alpha-ketoadipic acid, alpha-hydroxyadipic acid, alpha-aminoadipic acid, 1,2-butenedicarboxylic acid and elevation of plasma alpha-aminoadipic acid levels. The identity of these metabolites was established by various methods. The excretion of alpha-aminoadipic acid correlated to the lysine intake. Degradation studies with
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