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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

articles

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Survival after Treatment with Phenylacetate and Benzoate for Urea-Cycle Disorders.
G. M. Enns, S. A. Berry, G. T. Berry, W. J. Rhead, S. W. Brusilow, and A. Hamosh (2007)
N. Engl. J. Med. 356, 2282-2292
   Abstract »    Full Text »    PDF »
Alternative-Pathway Therapy for Hyperammonemia.
V. E. Shih (2007)
N. Engl. J. Med. 356, 2321-2322
   Full Text »    PDF »
Pharmacology Review: Sodium Phenylacetate and Sodium Benzoate in the Treatment of Neonatal Hyperammonemia.
A.-K. Niemi and G. M. Enns (2006)
NeoReviews 7, e486-e495
   Full Text »    PDF »
Clinical Consequences of Urea Cycle Enzyme Deficiencies and Potential Links to Arginine and Nitric Oxide Metabolism.
F. Scaglia, N. Brunetti-Pierri, S. Kleppe, J. Marini, S. Carter, P. Garlick, F. Jahoor, W. O'Brien, and B. Lee (2004)
J. Nutr. 134, 2775S-2782S
   Abstract »    Full Text »    PDF »
Quantification of Benzoic, Phenylacetic, and Phenylbutyric Acids from Filter-Paper Blood Spots by Gas Chromatography-Mass Spectrometry with Stable Isotope Dilution.
X. Yu, M. M. Thompson, D. Shi, and M. Tuchman (2001)
Clin. Chem. 47, 351-354
   Full Text »    PDF »
Induction of Apoptosis in Malignant B Cells by Phenylbutyrate or Phenylacetate in Combination with Chemotherapeutic Agents.
T. E. Witzig, M. Timm, M. Stenson, P. A. Svingen, and S. H. Kaufmann (2000)
Clin. Cancer Res. 6, 681-692
   Abstract »    Full Text »
Effect of Long-term Administration of Sodium Benzoate to a Patient with Partial Ornithine Carbamoyl Transferase Deficiency.
E. Takeda, Y. Kuroda, K. Toshima, T. Watanabe, E. Naito, and M. Miyao (1983)
Clinical Pediatrics 22, 206-208
   Abstract »    PDF »


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