Literature DB >> 19660971

Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.

Nicholas Ah Mew1, Irma Payan, Yevgeny Daikhin, Ilana Nissim, Itzhak Nissim, Mendel Tuchman, Marc Yudkoff.   

Abstract

We studied the effect on ureagenesis of a single dose of N-carbamylglutamate (NCG) in healthy young adults who received a constant infusion (300 min) of NaH(13)CO(3). Isotope ratio-mass spectrometry was used to measure the appearance of label in [(13)C]urea. At 90 min after initiating the H(13)CO3-infusion each subject took a single dose of NCG (50 mg/kg). In 5/6 studies the administration of NCG increased the formation of [(13)C]urea. Treatment with NCG significantly diminished the concentration of blood alanine, but not that of glutamine or arginine. The blood glucose concentration was unaffected by NCG administration. No untoward side effects were observed. The data indicate that treatment with NCG stimulates ureagenesis and could be useful in clinical settings of acute hyperammonemia of various etiologies.

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Year:  2009        PMID: 19660971      PMCID: PMC2784258          DOI: 10.1016/j.ymgme.2009.07.010

Source DB:  PubMed          Journal:  Mol Genet Metab        ISSN: 1096-7192            Impact factor:   4.797


  42 in total

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Authors:  S GRISOLIA; P P COHEN
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2.  In vivo nitrogen metabolism in ornithine transcarbamylase deficiency.

Authors:  M Yudkoff; Y Daikhin; I Nissim; A Jawad; J Wilson; M Batshaw
Journal:  J Clin Invest       Date:  1996-11-01       Impact factor: 14.808

3.  Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kinetics.

Authors:  T Yorifuji; J Muroi; A Uematsu; H Hiramatsu; T Momoi
Journal:  Hum Genet       Date:  1999-06       Impact factor: 4.132

Review 4.  The glucose-alanine cycle.

Authors:  P Felig
Journal:  Metabolism       Date:  1973-02       Impact factor: 8.694

5.  Reassessment of primed constant-infusion tracer method to measure urea kinetics.

Authors:  F Jahoor; R R Wolfe
Journal:  Am J Physiol       Date:  1987-04

6.  Role of acetylglutamate in ureotelism. Variations in acetylglutamate level and its possible significance in control of urea synthesis in mammalian liver.

Authors:  K Shigesada; K Aoyagi; M Tatibana
Journal:  Eur J Biochem       Date:  1978-04-17

7.  Propionic acidemia with severe hyperammonemia and defective glycine metabolism.

Authors:  T Shafai; L Sweetman; W Weyler; S I Goodman; P V Fennessey; W L Nyhan
Journal:  J Pediatr       Date:  1978-01       Impact factor: 4.406

8.  Cross-sectional multicenter study of patients with urea cycle disorders in the United States.

Authors:  Mendel Tuchman; Brendan Lee; Uta Lichter-Konecki; Marshall L Summar; Marc Yudkoff; Stephen D Cederbaum; Douglas S Kerr; George A Diaz; Margaretta R Seashore; Hye-Seung Lee; Robert J McCarter; Jeffrey P Krischer; Mark L Batshaw
Journal:  Mol Genet Metab       Date:  2008-06-17       Impact factor: 4.797

9.  Mitochondrial carbamyl phosphate and citrulline synthesis at high matrix acetylglutamate.

Authors:  N S Cohen; C W Cheung; F S Kyan; E E Jones; L Raijman
Journal:  J Biol Chem       Date:  1982-06-25       Impact factor: 5.157

Review 10.  Recent advances in the mechanism of pyridoxine-responsive disorders.

Authors:  B Fowler
Journal:  J Inherit Metab Dis       Date:  1985       Impact factor: 4.982

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  18 in total

1.  Measuring in vivo ureagenesis with stable isotopes.

Authors:  Marc Yudkoff; Nicholas Ah Mew; Yevgeny Daikhin; Oksana Horyn; Ilana Nissim; Itzhak Nissim; Irma Payan; Mendel Tuchman
Journal:  Mol Genet Metab       Date:  2010-02-26       Impact factor: 4.797

2.  N-carbamylglutamate enhancement of ureagenesis leads to discovery of a novel deleterious mutation in a newly defined enhancer of the NAGS gene and to effective therapy.

Authors:  Sandra K Heibel; Nicholas Ah Mew; Ljubica Caldovic; Yevgeny Daikhin; Marc Yudkoff; Mendel Tuchman
Journal:  Hum Mutat       Date:  2011-09-09       Impact factor: 4.878

3.  Precision medicine in rare disease: Mechanisms of disparate effects of N-carbamyl-l-glutamate on mutant CPS1 enzymes.

Authors:  Dashuang Shi; Gengxiang Zhao; Nicholas Ah Mew; Mendel Tuchman
Journal:  Mol Genet Metab       Date:  2016-12-08       Impact factor: 4.797

Review 4.  CPS1: Looking at an ancient enzyme in a modern light.

Authors:  Matthew Nitzahn; Gerald S Lipshutz
Journal:  Mol Genet Metab       Date:  2020-10-10       Impact factor: 4.797

5.  N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.

Authors:  Nicholas Ah Mew; Robert McCarter; Yevgeny Daikhin; Itzhak Nissim; Marc Yudkoff; Mendel Tuchman
Journal:  Pediatrics       Date:  2010-06-21       Impact factor: 7.124

6.  Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase.

Authors:  Itzhak Nissim; Oksana Horyn; Ilana Nissim; Yevgeny Daikhin; Ljubica Caldovic; Belen Barcelona; Javier Cervera; Mendel Tuchman; Marc Yudkoff
Journal:  J Biol Chem       Date:  2011-05-03       Impact factor: 5.157

Review 7.  N-acetylglutamate synthase: structure, function and defects.

Authors:  Ljubica Caldovic; Nicholas Ah Mew; Dashuang Shi; Hiroki Morizono; Marc Yudkoff; Mendel Tuchman
Journal:  Mol Genet Metab       Date:  2010-02-26       Impact factor: 4.797

Review 8.  Recurrent encephalopathy: NAGS (N-acetylglutamate synthase) deficiency in adults.

Authors:  A Cartagena; A N Prasad; C A Rupar; M Strong; M Tuchman; N Ah Mew; C Prasad
Journal:  Can J Neurol Sci       Date:  2013-01       Impact factor: 2.104

9.  Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate.

Authors:  Nicholas Ah Mew; Robert McCarter; Yevgeny Daikhin; Uta Lichter-Konecki; Ilana Nissim; Marc Yudkoff; Mendel Tuchman
Journal:  J Pediatr       Date:  2014-05-29       Impact factor: 4.406

10.  Efficacy of N-carbamoyl-L-glutamic acid for the treatment of inherited metabolic disorders.

Authors:  Cristel C Chapel-Crespo; George A Diaz; Kimihiko Oishi
Journal:  Expert Rev Endocrinol Metab       Date:  2016-09-28
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