Presku: | AAP51183 |
Size: | 100 ug |
Weight: | 58kDa |
Gene: | 162417 |
Format: | Lyophilized powder |
Target: | NAGS is a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia.The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia. |
Alternative names: | AGAS; ARGA; MGC133025 |