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Science 8 February 1980:
Vol. 207. no. 4431, pp. 659 - 661
DOI: 10.1126/science.6243418
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Articles
Science, Vol 207, Issue 4431, 659-661
Copyright © 1980 by American Association for the Advancement of Science
Amino acid acylation: a mechanism of nitrogen excretion in inborn errors of urea synthesis
S Brusilow,
J Tinker,
and
ML Batshaw
Treatment of a patient deficient in carbamyl phosphate synthetase with benzoate or phenylacetic acid resulted in an increase in urinary nitrogen, which could be accounted for by the respective amino acid acylation product, hippurate or phenylacetylgultamine. Benzoate treatment of four hyperammonemic comatose patients led to clinical improvement and a return of plasma ammonium levels toward normal.
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- Effect of Long-term Administration of Sodium Benzoate to a Patient with Partial Ornithine Carbamoyl Transferase Deficiency.
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Clinical Pediatrics
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