Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
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Crystal Structure of the N-Acetyltransferase Domain of Human N-Acetyl-L-Glutamate Synthase in Complex with N-Acetyl-L-Glutamate Provides Insights into Its Catalytic and Regulatory MechanismsMeasuring in vivo ureagenesis with stable isotopesN-acetylglutamate synthase: structure, function and defects.Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamateRecurrent encephalopathy: NAGS (N-acetylglutamate synthase) deficiency in adults.Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy.Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthaseN-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.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.New developments in the treatment of hyperammonemia: emerging use of carglumic acid.Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiencyClinical experience with N-carbamylglutamate in a single-centre cohort of patients with propionic and methylmalonic aciduria.First report of carglumic acid in a patient with citrullinemia type 1 (argininosuccinate synthetase deficiency).Challenges in optimizing sample preparation and LC-MS/MS conditions for the analysis of carglumic acid, an N-acetyl glutamate derivative in human plasma.Precision medicine in rare disease: Mechanisms of disparate effects of N-carbamyl-l-glutamate on mutant CPS1 enzymes.Pancreatic progenitor-derived hepatocytes are viable and functional in a 3D high density bioreactor culture system
P2860
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P2860
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
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article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 14 July 2009
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@en
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@nl
type
label
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@en
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@nl
prefLabel
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@en
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@nl
P2093
P2860
P1476
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis.
@en
P2093
Ilana Nissim
Irma Payan
Itzhak Nissim
Marc Yudkoff
Mendel Tuchman
Nicholas Ah Mew
Yevgeny Daikhin
P2860
P304
P356
10.1016/J.YMGME.2009.07.010
P577
2009-07-14T00:00:00Z