General Information:

Id: 5,454
Diseases: Metabolic
N-acetylglutamate synthase deficiency - [OMIM]
Homo sapiens
review
Reference: Haberle J(2011) Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency Ther Clin Risk Manag 7: 327-332 [PMID: 21941437]

Interaction Information:

Comment N-acetylglutamate synthase (NAGS) is localized in mitochondrion. NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 53225

gene/protein

NAGS

is localized in

cellular component

mitochondrion

in liver
Drugbank entries Show/Hide entries for NAGS
Comment Deficiency of N-acetylglutamate synthase belongs to the rare inborn errors and leads to hyperammonemia. (cited information)
Formal Description
Interaction-ID: 53226

gene/protein

NAGS

affects_activity of

disease

N-acetylglutamate synthase deficiency

Drugbank entries Show/Hide entries for NAGS
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as allosteric activator of carbamoylphosphate synthetase 1, the first enzyme of the urea cycle. (cited information)
Formal Description
Interaction-ID: 53227

gene/protein

NAGS

increases_activity of

process

urea cycle

Drugbank entries Show/Hide entries for NAGS
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as the allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 53228

gene/protein

NAGS

increases_quantity of

drug/chemical compound

N-Acetyl-L-glutamate

Drugbank entries Show/Hide entries for NAGS or N-Acetyl-L-glutamate
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as the allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 53229

gene/protein

NAGS

decreases_quantity of

drug/chemical compound

Glutamate

Drugbank entries Show/Hide entries for NAGS
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as the allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 53230

drug/chemical compound

N-Acetyl-L-glutamate

increases_activity of

gene/protein

CPS1

in mitochondria
Drugbank entries Show/Hide entries for N-Acetyl-L-glutamate or CPS1
Comment Carbamoylphosphate synthetase 1 is the first enzyme of the urea cycle and is localized in mitochondria. (cited information)
Formal Description
Interaction-ID: 53231

gene/protein

CPS1

is localized in

cellular component

mitochondrion

Drugbank entries Show/Hide entries for CPS1
Comment Carbamoylphosphate synthetase 1 is the first enzyme of the urea cycle and is localized in mitochondria. (cited information)
Formal Description
Interaction-ID: 53232

gene/protein

CPS1

increases_activity of

process

urea cycle

Drugbank entries Show/Hide entries for CPS1
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as the allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 53445

gene/protein

NAGS

decreases_quantity of

drug/chemical compound

Acetyl-CoA

Drugbank entries Show/Hide entries for NAGS
Comment NAGS catalyzes the conversion of glutamate and acetyl Co-enzyme A to N-acetylglutamate, which serves as the allosteric activator of the first enzyme of the urea cycle, carbamoylphosphate synthetase 1. (cited information)
Formal Description
Interaction-ID: 96305

drug/chemical compound

Glutamate

increases_quantity of

drug/chemical compound

N-Acetyl-L-glutamate

by N-acetylglutamate synthase (NAGS) in the presence of acetyl -CoA
Drugbank entries Show/Hide entries for