{"symbol":"NAGS","synonyms":["ARGA","AGAS"],"name":"N-acetylglutamate synthase","description":"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. [provided by RefSeq, Jul 2008]","ncbiEntrezGeneId":162417,"ncbiEntrezGeneUrl":"http://www.ncbi.nlm.nih.gov/gene/162417","proteins":[{"symbol":"NAGS_HUMAN","href":"/api/1.0/protein/NAGS_HUMAN"}],"hgncRootFamilies":[]}