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PMID: 6243418 Published · ppublish English Case Reports Journal Article Research Support, U.S. Gov't, P.H.S.

Amino acid acylation: a mechanism of nitrogen excretion in inborn errors of urea synthesis.

Science (New York, N.Y.) ·Vol. 207 ·No. 4431 ·1980-02-08 ·Pages 659-61

Brusilow S, Tinker J, Batshaw ML

Abstract

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.

MeSH Terms

Adolescent Amino Acid Metabolism, Inborn Errors/drug therapy Amino Acids/blood Ammonia/blood Benzoates/therapeutic use Carbamoyl-Phosphate Synthase (Ammonia)/deficiency Child Female Glutamine/metabolism Glycine/metabolism Hippurates/urine Humans Infant Male Nitrogen/blood Ornithine Carbamoyltransferase Deficiency Disease Phenylacetates/therapeutic use Phosphotransferases/deficiency

Chemicals

Amino Acids Benzoates Hippurates Phenylacetates Glutamine Ammonia Phosphotransferases Carbamoyl-Phosphate Synthase (Ammonia) Nitrogen Glycine

Authors & Affiliations

3 authors, click to expand affiliations / ORCID

Brusilow S

Tinker J

Batshaw M L

Article Info

Journal

Science (New York, N.Y.)

Abbr.

Science

ISSN

0036-8075

DOI

10.1126/science.6243418

Published

1980-02-08

Pages

659-61

Language

English

Region

United States

NLM ID

0404511

Subset

External Links

PubMed source DOI: 10.1126/science.6243418 View journal details

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Amino acid acylation: a mechanism of nitrogen excretion in inborn errors of urea synthesis.

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