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Formation of Hippuric Acid from Phenylalanine labelled with Carbon-14 in Phenylketonuric Subjects

Abstract

VARIOUS authors have recently demonstrated the formation of hippuric acid from endogenous benzoic acid in the mammalian organism. Fasting humans1 as well as humans and rats on a lactalbumin–carbohydrate diet2 excreted hippuric acid, and its formation in the intact rat could not be inhibited by sulphasuxidine2 or chloramphenicol3. Application of deuterium and phenylalanine labelled with carbon-14 resulted in labelled urinary hippuric acid2–4. In addition, when benzoic acid was isolated from the urine of human or rats fed benzoic acid, labelled with deuterium or carbon-14, the specific activity was about 20 per cent lower, indicating dilution by endogenous benzoic acid4,5. During the investigation of phenylalanine turnover in human phenylketonuria6, I had the opportunity of studying the formation of hippuric acid from phenylalanine. Phenylketonuric subjects are characterized by their inability to convert phenylalanine to tyrosine to any significant extent. This results in an accumulation of phenylalanine and its keto derivative, phenylpyruvic acid.

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GRÜMER, HD. Formation of Hippuric Acid from Phenylalanine labelled with Carbon-14 in Phenylketonuric Subjects. Nature 189, 63–64 (1961). https://doi.org/10.1038/189063a0

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