| Protein Name | N-acetylglutamate synthase |
|---|---|
| Organism | Homo sapiens |
| Gene ID | 162417 |
| Gene Symbol | NAGS |
| UniProt | Q8N159 (NAGS_HUMAN), Q2NKP2 (Q2NKP2_HUMAN) |
| Relationships |
Total Number of functionally related compound(s) :
44
Total Number of Articles : 59 |
Description |
N-acetylglutamate synthase |
| Gene Summary |
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] |
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