• Thumbnail for N-Acetylglutamate synthase
    N-Acetylglutamate synthase (NAGS) is an enzyme that catalyses the production of N-acetylglutamate (NAG) from glutamate and acetyl-CoA. Put simply NAGS...
    11 KB (1,223 words) - 06:49, 14 August 2023
  • N-acetylglutamate synthetase may refer to: Amino-acid N-acetyltransferase Glutamate N-acetyltransferase Urea cycle N-Acetylglutamate synthase This set...
    495 bytes (51 words) - 14:49, 2 August 2024
  • Thumbnail for N-Acetylglutamate synthase deficiency
    N-Acetylglutamate synthase deficiency is an autosomal recessive urea cycle disorder. The symptoms are visible within the first week of life and if not...
    5 KB (443 words) - 18:27, 26 October 2024
  • Thumbnail for N-Acetylglutamic acid
    acetyltransferase, and from glutamic acid and acetyl-CoA by the enzyme N-acetylglutamate synthase. The reverse reaction, hydrolysis of the acetyl group, is catalyzed...
    15 KB (1,318 words) - 18:44, 17 August 2022
  • activator of carbamoyl phosphate synthetase. Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is stimulated by both Arg, allosteric stimulator of NAGS...
    21 KB (2,219 words) - 13:28, 25 July 2024
  • N acetyltransferase deficiency N syndrome N-Acetylglutamate synthase deficiency N-acetyl-alpha-D-galactosaminidase N-acetyl-glucosamine-6-sulfate sulfatase...
    8 KB (654 words) - 05:37, 10 November 2024
  • Hyperammonemia develops due to the inhibitory effects of propionyl-CoA on N-acetylglutamate synthase, indirectly resulting in slowing of the urea cycle. Elevated metabolites...
    14 KB (1,383 words) - 14:38, 25 October 2024
  • Thumbnail for Carglumic acid
    Yudkoff M, Tuchman M (2004). "Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate". J Pediatr. 145 (4): 552–4. doi:10...
    7 KB (337 words) - 05:39, 10 April 2024
  • N-acetyl-L-glutamate synthetase may refer to: N-Acetylglutamate synthase Glutamate N-acetyltransferase This set index page lists enzyme articles associated...
    496 bytes (47 words) - 02:17, 17 October 2024
  • from acetyl-CoA at the N-α position; this prevents spontaneous cyclization. The enzyme N-acetylglutamate synthase (glutamate N-acetyltransferase) is responsible...
    61 KB (6,770 words) - 15:11, 20 August 2024
  • Thumbnail for Hyperammonemia
    Mendelian Inheritance in Man (OMIM): 237310 - hyperammonemia due to N-Acetylglutamate synthase deficiency Online Mendelian Inheritance in Man (OMIM): 237300...
    16 KB (1,589 words) - 11:16, 18 November 2024
  • Thumbnail for Allosteric regulation
    PMID 237460. Shi D, Allewell NM, Tuchman M (June 2015). "The N-Acetylglutamate Synthase Family: Structures, Function and Mechanisms". International Journal...
    37 KB (4,422 words) - 05:12, 29 September 2024
  • Thumbnail for Citrullinemia type I
    ornithine transcarbamylase deficiency, arginase deficiency, and N-Acetylglutamate synthase deficiency. Other diseases that may appear similar to CTLN1 include...
    5 KB (498 words) - 11:11, 13 August 2021
  • 613488; DDIT3 Myxoma, intracardiac; 255960; PRKAR1A N syndrome; 310465; POLA N-Acetylglutamate synthase deficiency; 237310; NAGS Naegeli–Franceschetti–Jadassohn...
    234 KB (18,877 words) - 06:16, 6 November 2024
  • Thumbnail for SLC22A5
    "Deficiency of the carnitine transporter (OCTN2) with partial N-acetylglutamate synthase (NAGS) deficiency". Journal of Inherited Metabolic Disease. 30...
    19 KB (2,294 words) - 13:22, 7 September 2023
  • Thumbnail for Carbamoyl phosphate synthetase
    1987). "Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate". The Biochemical Journal. 243 (1): 273–6...
    10 KB (923 words) - 10:40, 20 September 2024
  • NH4+. When amino acid catabolism increases, N-Acetylglutamate synthase is up-regulated, producing more N-acetyl-L-glutamate, which up-regulates carbamoyl...
    19 KB (1,853 words) - 16:05, 22 September 2024
  • 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...
    2 KB (169 words) - 17:24, 28 April 2024
  • is phosphorylated to give carbamoyl phosphate CPSI is regulated by N-acetylglutamate which acts as an obligate allosteric activator of CPS1. NAG, by binding...
    8 KB (823 words) - 10:11, 20 September 2024
  • 16, carbamoyl-phosphate synthase (ammonia) EC 2.7.2.6: formate kinase EC 2.7.2.7: butyrate kinase EC 2.7.2.8: acetylglutamate kinase EC 2.7.2.9: Now EC...
    215 KB (21,664 words) - 23:41, 8 May 2024