N-Acetylglutamate synthase, an early enzyme of the arginine pathway, provides acetylglutamate for ornithine synthesis in the so-called "acetylglutamate cycle." Because acetylglutamate is regenerated as ornithine is formed, the enzyme has only a catalytic or anaplerotic role in the pathway, ...
1) N-acetylglutamate synthase N-乙酰谷氨酸合成酶1. Objective This study was conducted to investigate the mechanism for the reduction of endogenous arginine synthesis by profiling the developmental expression pattern of intestinal N-acetylglutamate synthase(NAGS)in pre-weaned Tibetan piglets. 【目的】...
Caldovic L, Morizono H, Panglao M, Cheng S, Packman S, Tuchman M (2003). "Null mutations in the N-acetylglutamate synthase gene associated with acute neonatal disease and hyperammonemia".Hum Genet112(4): 364-8. PMID 12594532. Categories:Metabolic disorders|Inborn errors of metabolism ...
N-acetylglutamate synthase deficiency (NAGSD, MIM #237310) is an autosomal recessive disorder of the urea cycle that results from absent or decreased production of N-acetylglutamate (NAG) due to either decreased NAGS gene expression or defective NAGS enzyme. NAG is essential for the activity of...
1.Objective This study was conducted to investigate the mechanism for the reduction of endogenous arginine synthesis by profiling the developmental expression pattern of intestinal N-acetylglutamate synthase(NAGS)in pre-weaned Tibetan piglets.【目的】探讨藏猪仔猪肠道N-乙酰谷氨酸合成酶(NAGS)在哺乳期的表达...
-acetylglutamate synthase deficiencyUrea cycle defectHyperammonemiaCarbaglumic acidPrenatal diagnosis-acetyl glutamate synthase (NAGS) deficiency is the rarest urea cycle defect presenting as neonatal onset life-threatening hyperammonemia. We report here a family history of severe NAGS deficiency: after the ...
Arginine biosynthesis in Corynebacterium glutamicum consists of eight enzymatic steps, starting with acetylation of glutamate, catalysed by N-acetylglutamate synthase (NAGS). There are different kinds of known NAGSs, for example, “classical” ArgA, bifu
CPSIII increases in the presence of NAG. Abbreviations: NAGS – N-acetylglutamate synthase; NAG – N-acetylglutamate; CPSI – carbamylphosphate synthetase I; CPSIII – carbamylphosphate synthetase III; OTC – ornithine transcarbamylase; ASS – argininosuccinate synthase; ASL – argininosuccinate lyase...
Shi D, Caldovic L, Jin Z, Yu X, Qu Q, Roth L, Morizono H, Hathout Y, Allewell NM, Tuchman M (2006) Expression, crystallization and preliminary crystallographic studies of a novel bifunctional N-acetylglutamate synthase/kinase from Xanthomonas campestris homologous to vertebrate N-acetyl...
The effects of dietary conditions on the degradation of hepatic N-acetyl-L-glutamate, an essential activator of carbamoyl phosphate synthase I [EC 6.3.4.16], were studied by injecting mice with [14C]glutamate and by following the fate of labeled N-acetyl-L-glutamate. In fasted mice, the radi...