@article{48a1ec1436f246a987792a4161b4012b,
title = "NEW PATHWAYS OF NITROGEN EXCRETION IN INBORN ERRORS OF UREA SYNTHESIS",
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.",
author = "Brusilow, {Saul W.} and Valle, {David L.} and Batshaw, {Mark L.}",
note = "Funding Information: suggest that research be directed to finding alternative pathways of waste nitrogen excretion. The examples cited here suggest that urea need not be the principle end-product of nitrogen metabolism in man but that citrulline, argininosuccinic acid, arginine, hippuric acid, and phenylacetylglutamine might also serve this func- tion and thus be added to the list of products used by animals to excrete waste nitrogen which already in- cludes ammonium, urea, uric acid, allantoin, and allan- toic acid. These studies were supported by grants from the U.S.P.H.S. (ROI-HDlI134) (POI-AMI8020) (ROI-AM20328) (K07-NS500342) (MOI-RROO052) and the National Foundation. D.L.V. is an investiga-tor of the Howard Hughes Institute. We are grateful for the suggestion urea-cycle enzymopathies.of Dr Norman Radin that acylating agents might be useful in treating",
year = "1979",
month = sep,
day = "1",
doi = "10.1016/S0140-6736(79)91503-4",
language = "English (US)",
volume = "314",
pages = "452--454",
journal = "The Lancet",
issn = "0140-6736",
publisher = "Elsevier Limited",
number = "8140",
}