N-Acetylglutamate synthase

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.

This transferase article is a stub. You can help Wikipedia by expanding it.

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 - Serine C-palmitoyltransferase (SPTLC1, SPTLC2) - HGSNAT

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

v • d • e Mitochondrial enzymes and transporters

Outer membrane	fatty acid degradation (Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase) tryptophan metabolism (Kynureninase) monoamine neurotransmitter metabolism (Monoamine oxidase)

Intermembrane space	Adenylate kinase - Creatine kinase

Inner membrane	oxidative phosphorylation (Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase) pyrimidine metabolism (Dihydroorotate dehydrogenase) mitochondrial shuttle (Malate-aspartate shuttle, Glycerol phosphate shuttle) other (Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II)

Matrix	citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase) anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex) urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase) alcohol metabolism (ALDH2)

Other/to be sorted	Cholesterol side-chain cleavage enzyme

Mitochondrial DNA	Complex I (7 units) - Complex III (1 unit) - Complex IV (3 units) - ATP synthase (2 units)

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N-Acetylglutamate synthase

Clinical significance

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