N-Acetylglutamate synthase

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N-Acetylglutamate synthase
Identifiers
Symbol NAGS
HUGO 17996
Entrez 162417
OMIM 608300
RefSeq NM_153006
UniProt Q8N159
Other data
EC number 2.3.1.1
Locus Chr. 17 q21.31

N-acetylglutamate synthase is an enzyme which catalyses the production of N-acetylglutamate from acetyl-CoA and glutamate.

This enzyme is important in bacteria and plants for the synthesis of arginine, because it is part of this pathway.

Also this enzyme is important for mammals, because it produces the regulator of urea cycle N-acetylglutamate, which activates carbamoyl phosphate synthetase I, which catalyses the initial reactions of urea cycle.

[edit] Clinical significance

Mutations in the gene are associated with N-Acetylglutamate synthase deficiency.

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[edit] References

  • Lehninger principles of biochemistry, 4th edition, David L. Nelson, Michael M. Cox

[edit] External links

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Transferases: acyltransferases (EC 2.3)

Carnitine O-palmitoyltransferase - Glyceronephosphate O-acyltransferase - Lecithin-cholesterol acyltransferase - Citrate synthase

Acetyltransferases: Beta-galactoside transacetylase - Choline acetyltransferase - Chloramphenicol acetyltransferase - Histone acetyltransferase (P300/CBP)- N-Acetylglutamate synthase -

Serotonin N-acetyl transferase

Aminoacyltransferases: Peptidyl transferase - Transglutaminase (Tissue transglutaminase, Factor XIII)

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