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

Stable Identifier
R-HSA-70542
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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glutamate + acetyl CoA => N-acetyl glutamate + CoA
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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
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
Participants
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Output
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as an event of
Catalyst Activity

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

Physical Entity
NAGS(19-534) [mitochondrial matrix] (Homo sapiens)
Activity
L-glutamate N-acetyltransferase activity (GO:0004042)
This event is regulated
Positively by
Regulator
L-Arg [mitochondrial matrix]
Orthologous Events
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Bos taurus)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Canis familiaris)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Danio rerio)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Dictyostelium discoideum)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Mus musculus)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Rattus norvegicus)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Saccharomyces cerevisiae)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Schizosaccharomyces pombe)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Sus scrofa)
glutamate + acetyl CoA => N-acetyl glutamate + CoA (Xenopus tropicalis)
Cross References
Rhea
Authored
D'Eustachio, P  (2003-06-24)
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