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CAS No: 1188-38-1 Catalog No: AG003868 MDL No:MFCD00047874
Title | Journal |
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Effects of dietary L-arginine or N-carbamylglutamate supplementation during late gestation of sows on the miR-15b/16, miR-221/222, VEGFA and eNOS expression in umbilical vein. | Amino acids 20120601 |
A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase. | Molecular genetics and metabolism 20120601 |
Effect of dietary arginine and N-carbamoylglutamate supplementation on reproduction and gene expression of eNOS, VEGFA and PlGF1 in placenta in late pregnancy of sows. | Animal reproduction science 20120601 |
N-carbamylglutamate enhances pregnancy outcome in rats through activation of the PI3K/PKB/mTOR signaling pathway. | PloS one 20120101 |
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. | Human mutation 20111001 |
The HepaRG cell line is suitable for bioartificial liver application. | The international journal of biochemistry & cell biology 20111001 |
Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase. | The Journal of biological chemistry 20110624 |
N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia. | European journal of pediatrics 20110601 |
New developments in the treatment of hyperammonemia: emerging use of carglumic acid. | International journal of general medicine 20110101 |
MR spectroscopy-based brain metabolite profiling in propionic acidaemia: metabolic changes in the basal ganglia during acute decompensation and effect of liver transplantation. | Orphanet journal of rare diseases 20110101 |
Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency. | Therapeutics and clinical risk management 20110101 |
Gateways to clinical trials. | Methods and findings in experimental and clinical pharmacology 20101201 |
Dietary supplementation with L-arginine or N-carbamylglutamate enhances intestinal growth and heat shock protein-70 expression in weanling pigs fed a corn- and soybean meal-based diet. | Amino acids 20100801 |
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. | Pediatrics 20100701 |
Carglumic acid approved to treat genetic hyperammonemia. | American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists 20100501 |
Outpatient treatment of propionic acidemia-associated hyperammonemia with N-carbamoyl-L-glutamate in an infant. | Clinical therapeutics 20100401 |
Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency. | European journal of pediatrics 20100201 |
Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia. | European journal of pediatrics 20100101 |
N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria. | Neonatology 20100101 |
N-acetylglutamate synthase: structure, function and defects. | Molecular genetics and metabolism 20100101 |
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis. | Molecular genetics and metabolism 20091201 |
[N-carbamyl glutamate treatment in hyperammoniemia decompensated propionic acidaemia]. | Anales de pediatria (Barcelona, Spain : 2003) 20091201 |
The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease. | Metabolic brain disease 20090901 |
Pricing of orphan drugs. | Lancet (London, England) 20090207 |
Uncovering metabolic pathways relevant to phenotypic traits of microbial genomes. | Genome biology 20090101 |
N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia. | Journal of inherited metabolic disease 20081201 |
N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers. | Pediatric research 20080801 |
[A newborn infant with hyperventilation]. | Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 20080626 |
Carglumic acid: a second look. Confirmed progress in a rare urea cycle disorder. | Prescrire international 20080401 |
Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia? | Orphanet journal of rare diseases 20080101 |
Mutations and polymorphisms in the human N-acetylglutamate synthase (NAGS) gene. | Human mutation 20070801 |
A trial with N-carbamylglutamate may not detect all patients with NAGS deficiency and neonatal onset. | Journal of inherited metabolic disease 20070601 |
Metabolic fates of ammonia-N in ruminal epithelial and duodenal mucosal cells isolated from growing sheep. | Journal of dairy science 20051101 |
Neonatal hyperammonemia: the N-carbamoyl-L-glutamic acid test. | The Journal of pediatrics 20050801 |
Misleading diagnosis of partial N-acetylglutamate synthase deficiency based on enzyme measurement corrected by mutation analysis. | Acta paediatrica (Oslo, Norway : 1992) 20050101 |
N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia. | Journal of inherited metabolic disease 20050101 |
Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. | The Journal of pediatrics 20041001 |
Urea synthesis by ruminal epithelial and duodenal mucosal cells from growing sheep. | Journal of dairy science 20040601 |
Mammalian N-acetylglutamate synthase. | Molecular genetics and metabolism 20040401 |
Carglumic acid: new preparation. An advance in rare urea cycle disorders. | Prescrire international 20040201 |
Hyperammonaemia as a cause of psychosis in an adolescent. | European journal of pediatrics 20031101 |
N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria. | Molecular genetics and metabolism 20030801 |
N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy. | Annals of neurology 20021201 |
Mixture of N-carbamoyl-L-glutamate plus L-arginine can protect rats with liver cirrhosis from acute ammonia intoxication. | Journal of hepatology 20011201 |
Hyperinsulinism and hyperammonemia syndrome: report of twelve unrelated patients. | Pediatric research 20010901 |
[Orphan drugs and metabolic disorders]. | Revista de neurologia 20010801 |
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