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

Stable Identifier
R-HSA-70542
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
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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
12459178 Cloning and expression of the human N-acetylglutamate synthase gene.

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

Biochem Biophys Res Commun 2002
15050968 Mammalian N-acetylglutamate synthase

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

Mol Genet Metab 2004
Participants
Participant Of
hasEvent
Catalyst Activity
Catalyst Activity
Title
acetyl-CoA:L-glutamate N-acetyltransferase activity of NAGS(19-534) [mitochondrial matrix]
Physical Entity
Activity
This event is regulated
Orthologous Events
Cross References
Rhea
Authored