N-Acetylglutamate synthase

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N-Acetylglutamate synthase
Identifiers
Symbol	NAGS
Entrez	162417
HUGO	17996
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.

Clinical significance

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

Template:Enzyme-stub

References

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

External links

• MeSH N-Acetylglutamate+Synthase

v • d • e Transferases: acyltransferases (EC 2.3)
2.3.1: other than amino-acyl groups (mostly acetyltransferases)	N-Acetylglutamate synthase - Choline acetyltransferase - Acetyl-Coenzyme A acetyltransferase - Dihydrolipoyl transacetylase - Acetyl-CoA C-acyltransferase - Beta-galactoside transacetylase - Carnitine O-palmitoyltransferase (CPT1, CPT2) - Acyltransferase like 2 - Chloramphenicol acetyltransferase - Aminolevulinic acid synthase - Beta-ketoacyl-ACP synthase - Glyceronephosphate O-acyltransferase - Lecithin-cholesterol acyltransferase - Histone acetyltransferase (P300/CBP) - Serotonin N-acetyl transferase
2.3.2 - Aminoacyltransferases	Gamma glutamyl transpeptidase - Peptidyl transferase - Transglutaminase (Tissue transglutaminase, Keratinocyte transglutaminase, Factor XIII)
2.3.3 - converted into alkyl on transfer	Citrate synthase - ATP citrate lyase - HMG-CoA synthase

v • d • e Metabolism: amino acid metabolism - urea cycle enzymes
Main cycle	mitochondrial matrix: Carbamoyl phosphate synthetase I - Ornithine transcarbamylase cytosol: Argininosuccinate synthetase - Argininosuccinate lyase - Arginase
Regulatory/transport	N-Acetylglutamate synthase - Ornithine translocase
see also disorders

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .