Journal ArticleDOI
New pathways of nitrogen excretion in inborn errors of urea synthesis
TLDR
The urea cycle may serve as a model for the development of excretion pathways of toxic precursors which accumulate in inborn errors of metabolism.About:
This article is published in The Lancet.The article was published on 1979-09-01. It has received 176 citations till now. The article focuses on the topics: Argininosuccinic acid & Urea cycle.read more
Citations
More filters
Journal ArticleDOI
Survival after treatment with phenylacetate and benzoate for urea-cycle disorders
TL;DR: In this article, the authors reported the results of a 25-year, open-label, uncontrolled study of sodium phenylacetate and sodium benzoate therapy (Ammonul, Ucyclyd Pharma) in 299 patients with urea-cycle disorders in whom there were 1181 episodes of acute hyperammonemia.
Journal ArticleDOI
Urea cycle disorders
J.V. Leonard,A.A.M. Morris +1 more
TL;DR: Most patients with urea cycle disorders who present as neonates, do so with deteriorating feeding, drowsiness and tachypnoea, following a short initial period when they appear well, and the aim of treatment is to lower the ammonia concentrations as fast as possible.
Journal ArticleDOI
Alternative pathway therapy for urea cycle disorders: twenty years later.
TL;DR: It must be acknowledged that alternative pathway therapy has limited effectiveness in preventing hyperammonemia and must be combined with effective dietary management, and in children with neonatal-onset disease or in those with very poor metabolic control, liver transplantation should be considered.
Journal ArticleDOI
Ammonia toxicity to the brain.
TL;DR: Several potential neuroprotective strategies have been put forward recently, including the use of NMDA receptor antagonists, nitric oxide inhibitors, creatine, acetyl-L-carnitine, CNTF or inhibitors of MAPKs and glutamine synthetase.
Journal ArticleDOI
Current strategies for the management of neonatal urea cycle disorders
TL;DR: The methods outlined here should help treat a patient by drawing on the experience of others who have treated patients with urea cycle disorders, and do not purport to be the final word in treating children with these disorders.
References
More filters
Journal ArticleDOI
The conjugation of phenylacetic acid in man, sub-human primates and some non-primate species.
TL;DR: The conjugation of phenylacetic acid with taurine is reported for the first time and occurs in all the species examined except the vampire bat and domestic hen, but its quantitative occurrence is haphazard amongst thespecies examined.
Journal ArticleDOI
Synthesis of phenylacetylglutamine by human tissue.
Kivie Moldave,Alton Meister +1 more
Journal ArticleDOI
Renal clearances of amino acids in normal adults and in patients with aminoaciduria.
D. C. Cusworth,C. E. Dent +1 more
TL;DR: The studies reported here were undertaken to confirm or otherwise previous theories of the mechanism of amino acids excretion and to elucidate further the mode of amino acid excretion in certain metabolic diseases.
Journal ArticleDOI
The requirement for coenzyme A in the enzymatic synthesis of hippuric acid.
TL;DR: The experiments reported in the present paper show that benzoyl phosphate is not the high energy intermediate in hippuric acid synthesis, and further that coenzyme A is required for the condensation of benzoic acid with glycine.
Journal ArticleDOI
Identification of separate acyl- CoA:glycine and acyl-CoA:L-glutamine N-acyltransferase activities in mitochondrial fractions from liver of rhesus monkey and man.
TL;DR: Additional studies with the monkey liver enzymes revealed that acyl-CoA substrates for one transferase inhibit the other, that the apparent Km value is low (10(-6) to 10(-5) M range) for the preferred acyl CoA substrate as compared to the amino acid acceptor (greater than 10(-2) M) and that both transferases have a molecular weight of approximately 24,000.