glutamate + acetyl CoA => N-acetyl glutamate + CoA

Stable Identifier
Reaction [transition]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Mitochondrial N acetylglutamate synthetase (NAGS) catalyzes the reaction of glutamate and acetyl-CoA to form N-acetylglutamate and CoA. NAGS is activated by arginine and the N-acetylglutamate produced in the reaction in turn is required to activate carbamoyl synthetase I. Consistent with this regulatory role in urea synthesis, NAGS mutations in humans are associated with hyperammonemia (Caldovic et al. 2002; Morizono et al. 2004).

Literature References
PubMed ID Title Journal Year
15050968 Mammalian N-acetylglutamate synthase

Tuchman, M, Shi, D, Caldovic, L, Morizono, H

Mol Genet Metab 2004
12459178 Cloning and expression of the human N-acetylglutamate synthase gene.

Tuchman, M, Malamy, MH, Allewell, NM, Yu, X, Shi, D, Gracia Panglao, M, Gallegos, R, Caldovic, L, Morizono, H

Biochem Biophys Res Commun 2002
Catalyst Activity

acetyl-CoA:L-glutamate N-acetyltransferase activity of NAGS(19-534) [mitochondrial matrix]

This event is regulated
Positively by