Effects of ascorbic acid in alkaptonuria: Alterations in benzoquinone acetic acid and an ontogenic effect in infancy

Jon A. Wolff, Bruce Barshop, William L. Nyhan, Jack Leslie, J. E. Seegmiller, Harry Gruber, Michael Garst, Susan Winter, Kimberlee Michals, Reuben Matalon

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76 Scopus citations

Abstract

The effects of ascorbic acid on the excretion of homogentisic acid and its derivative benzoquinone acetic acid were studied in two adults and three infants. The administration of relatively large amounts of ascorbic acid to the adults was followed by a disappearance of benzoquinone acetic acid from the urine, whereas the level of excretion of homogentisic acid did not change. This could have relevance to the pathogenesis of ochronotic arthritis. In the 4-mo-old infant and the 5-mo-old infant ascorbic acid in the urine may have doubled the amount of homogentisic acid, presumably through an effect on the immature P-hydroxyphenylpyruvic acid oxidase. Dietary reduction of the intake of tyrosine and phenylalanine substantially reduced the excretion of homogentisic acid.

Original languageEnglish (US)
Pages (from-to)140-144
Number of pages5
JournalPediatric Research
Volume26
Issue number2
DOIs
StatePublished - Aug 1989
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Wolff, J. A., Barshop, B., Nyhan, W. L., Leslie, J., Seegmiller, J. E., Gruber, H., Garst, M., Winter, S., Michals, K., & Matalon, R. (1989). Effects of ascorbic acid in alkaptonuria: Alterations in benzoquinone acetic acid and an ontogenic effect in infancy. Pediatric Research, 26(2), 140-144. https://doi.org/10.1203/00006450-198908000-00015