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N-Acetylglutamate Synthase (NAGS)

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AGAS; ARGA; NAT7; Amino-acid acetyltransferase

N-Acetylglutamate Synthase (NAGS)
CCC G244
HGNC 17996
MGI 2387600
PubMed 12447942
RGD 1565783
UniProt Q8N159
Wiki NAGS
The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia.
NAGS gene, which encodes a deduced 534-amino acid protein with a molecular mass of 58.1 kD. NAGS contains an N-terminal mitochondrial targeting signal, a variable domain, and a 440-amino acid C-terminal conserved domain that shares 92% identity with the conserved domain of mouse Nags.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPG244Hu01 Recombinant N-Acetylglutamate Synthase (NAGS) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to N-Acetylglutamate Synthase (NAGS) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to N-Acetylglutamate Synthase (NAGS) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for N-Acetylglutamate Synthase (NAGS) CLIA Kit Customized Service Offer
n/a ELISA Kit for N-Acetylglutamate Synthase (NAGS) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPG244Mu01 Recombinant N-Acetylglutamate Synthase (NAGS) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to N-Acetylglutamate Synthase (NAGS) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to N-Acetylglutamate Synthase (NAGS) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for N-Acetylglutamate Synthase (NAGS) CLIA Kit Customized Service Offer
n/a ELISA Kit for N-Acetylglutamate Synthase (NAGS) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant N-Acetylglutamate Synthase (NAGS) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to N-Acetylglutamate Synthase (NAGS) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to N-Acetylglutamate Synthase (NAGS) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for N-Acetylglutamate Synthase (NAGS) CLIA Kit Customized Service Offer
n/a ELISA Kit for N-Acetylglutamate Synthase (NAGS) ELISA Kit Customized Service Offer
  1. "Mutation analysis in patients with N-acetylglutamate synthase deficiency."Hum. Mutat. 21:593-597(2003) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "Cloning and expression of the human N-acetylglutamate synthase gene."Biochem. Biophys. Res. Commun. 299:581-586(2002) [PubMed] [Europe PMC] [Abstract]
  4. "Crystal structure of the N-acetyltransferase domain of human N-acetyl-L-glutamate synthase in complex with N-acetyl-L-glutamate provides insights into its catalytic and regulatory mechanisms."PLoS ONE 8:E70369-E70369(2013) [PubMed] [Europe PMC] [Abstract]
  5. "Identification of novel mutations of the human N-acetylglutamate synthase gene and their functional investigation by expression studies."Biochim. Biophys. Acta 1740:54-59(2005) [PubMed] [Europe PMC] [Abstract]