|1.||de Almeida, Isabel Tavares: 1 article (08/2011)|
|2.||Duran, Marinus: 1 article (08/2011)|
|3.||van Cruchten, Arno: 1 article (08/2011)|
|4.||Ijlst, Lodewijk: 1 article (08/2011)|
|5.||Silva, Margarida F B: 1 article (08/2011)|
|6.||Wanders, Ronald J A: 1 article (08/2011)|
|7.||Aires, Cátia C P: 1 article (08/2011)|
|8.||Häberle, Johannes: 1 article (01/2011)|
|9.||Nissim, Itzhak: 1 article (08/2008)|
|10.||Caldovic, Ljubica: 1 article (08/2008)|
01/01/2011 - "The product of NAGS is N-acetylglutamate which is the absolutely required allosteric activator of the first urea cycle enzyme carbamoylphosphate synthetase 1. In defects of NAGS, the urea cycle function can be severely affected resulting in fatal hyperammonemia in neonatal patients or at any later stage in life. "
06/15/1987 - "In hyperammonemia, urea production and N-acetylglutamate levels were decreased by sodium benzoate. "
12/01/1979 - "A decreased level of N-acetylglutamate in liver mitochondria that would follow inhibition of N-acetylglutamate synthetase by propionyl-CoA would be expected to lead to hyperammonemia. "
08/01/2011 - "To elucidate the mechanisms which underlie VPA-induced hyperammonemia, the aim of this study was to evaluate the effect of both VPA and its reactive intermediate, valproyl-CoA (VP-CoA), on the synthesis of N-acetylglutamate (NAG), a prime metabolite activator of the urea cycle. "
04/01/1984 - "Valproate-induced hyperammonemia is probably the result of depletion of mitochondrial acetyl CoA and decreased production of N-acetylglutamate, the obligatory activator of the first enzyme of the urea cycle, carbamyl phosphate synthetase I. Anticonvulsant-mediated microsomal enzyme induction may also contribute."
08/01/2008 - "N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers."
01/01/2005 - "In patients with propionic aciduria, the accumulating metabolite propionyl-CoA causes a disturbance of the urea cycle via the inhibition of N-acetylglutamate synthesis. "
|3.||Ornithine Carbamoyltransferase Deficiency Disease
08/01/1990 - "Hepatic N-acetylglutamate content in 3 patients with complete ornithine transcarbamylase deficiency was below the normal mean but within 2 standard deviations from the mean (8.5-12.8 nmol/g) whereas it was higher (30.7 nmol/g) in another partial ornithine transcarbamylase deficient patient. "
04/01/1990 - "N-acetylglutamate content in liver and gut of normal and fasted mice, normal human livers, and livers of individuals with carbamyl phosphate synthetase or ornithine transcarbamylase deficiency."
|4.||Canavan Disease (Leukodystrophy, Spongiform)
|5.||Wounds and Injuries (Trauma)
|2.||Coenzyme A (CoA)
|5.||Carbamyl Phosphate (Carbamoyl Phosphate)
|6.||propionyl-coenzyme A (propionyl-CoA)
|7.||Amino-Acid N-Acetyltransferase (N-Acetylglutamate Synthase)
|9.||Biological Markers (Surrogate Marker)
|10.||Valproic Acid (Valproate, Semisodium)
|1.||Protein-Restricted Diet (Diet, Protein Restricted)