NAGS (N-acetylglutamate synthase)

2014-11-01  

Identity

HGNC
LOCATION
17q21.31
LOCUSID
ALIAS
AGAS,ARGA
FUSION GENES

Other Information

Locus ID:

NCBI: 162417
MIM: 608300
HGNC: 17996
Ensembl: ENSG00000161653

Variants:

dbSNP: 162417
ClinVar: 162417
TCGA: ENSG00000161653
COSMIC: NAGS

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000161653ENST00000293404Q8N159
ENSG00000161653ENST00000589767K7EK11

Expression (GTEx)

0
5
10
15
20
25
30
35
40

Pathways

PathwaySourceExternal ID
Arginine biosynthesisKEGGko00220
Arginine biosynthesisKEGGhsa00220
Metabolic pathwaysKEGGhsa01100
2-Oxocarboxylic acid metabolismKEGGhsa01210
2-Oxocarboxylic acid metabolismKEGGko01210
Biosynthesis of amino acidsKEGGhsa01230
Biosynthesis of amino acidsKEGGko01230
MetabolismREACTOMER-HSA-1430728
Metabolism of amino acids and derivativesREACTOMER-HSA-71291
Urea cycleREACTOMER-HSA-70635
Metabolism of polyaminesREACTOMER-HSA-351202

PharmGKB

Entity IDNameTypeEvidenceAssociationPKPDPMIDs
PA165958402carglumic acidChemicalLabelAnnotationassociated
PA166176020sodium benzoate / sodium phenylacetateChemicalLabelAnnotationassociated

References

Pubmed IDYearTitleCitations
370477262023NAGS, CPS1, and SLC25A13 (Citrin) at the Crossroads of Arginine and Pyrimidines Metabolism in Tumor Cells.0
370477262023NAGS, CPS1, and SLC25A13 (Citrin) at the Crossroads of Arginine and Pyrimidines Metabolism in Tumor Cells.0
345106282021Noncoding sequence variants define a novel regulatory element in the first intron of the N-acetylglutamate synthase gene.2
345106282021Noncoding sequence variants define a novel regulatory element in the first intron of the N-acetylglutamate synthase gene.2
303375522018N-Acetylglutamate Synthase Deficiency Due to a Recurrent Sequence Variant in the N-acetylglutamate Synthase Enhancer Region.5
303375522018N-Acetylglutamate Synthase Deficiency Due to a Recurrent Sequence Variant in the N-acetylglutamate Synthase Enhancer Region.5
270374982016Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations.16
277712892016A novel UPLC-MS/MS based method to determine the activity of N-acetylglutamate synthase in liver tissue.2
270374982016Understanding N-Acetyl-L-Glutamate Synthase Deficiency: Mutational Spectrum, Impact of Clinical Mutations on Enzyme Functionality, and Structural Considerations.16
277712892016A novel UPLC-MS/MS based method to determine the activity of N-acetylglutamate synthase in liver tissue.2
236437122014Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters.19
236437122014Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters.19
223839522012Transcriptional regulation of N-acetylglutamate synthase.10
223839522012Transcriptional regulation of N-acetylglutamate synthase.10
195331692010Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency.21

Citation

Dessen P

NAGS (N-acetylglutamate synthase)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/70677/nags-(n-acetylglutamate-synthase)