NEW PATHWAYS OF NITROGEN EXCRETION IN INBORN ERRORS OF UREA SYNTHESIS

Saul W. Brusilow, David L. Valle, Mark L. Batshaw

Research output: Contribution to journalShort survey

Abstract

The defect in nitrogen excretion in patients with inborn errors of urea synthesis can be controlled by exploiting the biosynthetic pathways of readily excretable non-urea metabolites which contain nitrogen derived from ammonium, alanine, glutamate, and glutamine. Two classes of such metabolites are the urea-cycle intermediates-including citrulline, argininosuccinic acid, and arginine-and the aminoacid acylation products-hippuric acid (the glycine conjugate of benzoic acid) and phenylactylglutamine (the glutamine conjugate of phenylactic acid). Thus the urea cycle may serve as a model for the development of excretion pathways of toxic precursors which accumulate in inborn errors of metabolism.

Original languageEnglish (US)
Pages (from-to)452-454
Number of pages3
JournalThe Lancet
Volume314
Issue number8140
DOIs
StatePublished - Sep 1 1979

ASJC Scopus subject areas

  • Medicine(all)

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