N-Acetylglutamate synthase (NAGS) is an enzyme that catalyses the production of N-acetylglutamate (NAG) from glutamate and acetyl-CoA. Put simply NAGS...

N-acetylglutamate synthetase may refer to: Amino-acid N-acetyltransferase Glutamate N-acetyltransferase Urea cycle N-Acetylglutamate synthase This set...

N-Acetylglutamate synthase deficiency is an autosomal recessive urea cycle disorder. The symptoms are visible within the first week of life and if not...

acetyltransferase, and from glutamic acid and acetyl-CoA by the enzyme N-acetylglutamate synthase. The reverse reaction, hydrolysis of the acetyl group, is catalyzed...

activator of carbamoyl phosphate synthetase. Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is stimulated by both Arg, allosteric stimulator of NAGS...

N acetyltransferase deficiency N syndrome N-Acetylglutamate synthase deficiency N-acetyl-alpha-D-galactosaminidase N-acetyl-glucosamine-6-sulfate sulfatase...

Hyperammonemia develops due to the inhibitory effects of propionyl-CoA on N-acetylglutamate synthase, indirectly resulting in slowing of the urea cycle. Elevated metabolites...

Yudkoff M, Tuchman M (2004). "Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate". J Pediatr. 145 (4): 552–4. doi:10...

Mendelian Inheritance in Man (OMIM): 237310 - hyperammonemia due to N-Acetylglutamate synthase deficiency Online Mendelian Inheritance in Man (OMIM): 237300...

ornithine transcarbamylase deficiency, arginase deficiency, and N-Acetylglutamate synthase deficiency. Other diseases that may appear similar to CTLN1 include...

PMID 237460. Shi D, Allewell NM, Tuchman M (June 2015). "The N-Acetylglutamate Synthase Family: Structures, Function and Mechanisms". International Journal...

from acetyl-CoA at the N-α position; this prevents spontaneous cyclization. The enzyme N-acetylglutamate synthase (glutamate N-acetyltransferase) is responsible...

1987). "Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate". The Biochemical Journal. 243 (1): 273–6...

In enzymology, a glutamate N-acetyltransferase (EC 2.3.1.35) is an enzyme that catalyzes the chemical reaction N2-acetyl-L-ornithine + L-glutamate ⇌ {\displaystyle...

NH4+. When amino acid catabolism increases, N-Acetylglutamate synthase is up-regulated, producing more N-acetyl-L-glutamate, which up-regulates carbamoyl...

613488; DDIT3 Myxoma, intracardiac; 255960; PRKAR1A N syndrome; 310465; POLA N-Acetylglutamate synthase deficiency; 237310; NAGS Naegeli–Franceschetti–Jadassohn...

is phosphorylated to give carbamoyl phosphate CPSI is regulated by N-acetylglutamate which acts as an obligate allosteric activator of CPS1. NAG, by binding...

O-methyltransferase EC 2.1.1.16: methylene-fatty-acyl-phospholipid synthase EC 2.1.1.17: phosphatidylethanolamine N-methyltransferase EC 2.1.1.18: polysaccharide O-methyltransferase...

"Deficiency of the carnitine transporter (OCTN2) with partial N-acetylglutamate synthase (NAGS) deficiency". Journal of Inherited Metabolic Disease. 30...