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0NAGS_HUMAN*   SwissProt (?) | Description Local Annotation Link Reference
General Information
NameNAGS
DescriptionN-acetylglutamate synthase, mitochondrial precursor (ec 2.3.1.1) (amino-acid acetyltransferase) .
SpeciesHomo sapiens (NCBI taxonomy ID: 9606)
GON/A
Domain Architecture (Details)
InterPro domains unassigned to SynO:
This region of unknown function is found at the C terminus of Neurospora crassa acetylglutamate synthase (). It is also found C-terminal to the amino acid kinase region in some fungal acetylglutamate kinase enzymes (). These enzymes play a role in arginine biosynthesis.
  IPR006855:Protein of unknown function DUF619
This entry contains proteins with various specificities and includes the aspartate.lutamate and uridylate kinase families. In prokaryotes and plants the synthesis of the essential amino acids lysine and threonine is predominantly regulated by feed-back inhibition of aspartate kinase (AK) and dihydrodipicolinate synthase (DHPS).In Escherichia coli.hrA.etLM.nd lysC encode aspartokinase isozymes that show feedback inhibition by threonine.ethionine.nd lysine.espectively . The lysine-sensitive isoenzyme of aspartate kinase from spinach leaves has a subunit composition of 4 large and 4 small subunits . In plants although the control of carbon fixation and nitrogen assimilation has been studied in detail.elatively little is known about the regulation of carbon and nitrogen flow into amino acids. The metabolic regulation of expression of an Arabidopsis thaliana aspartate kinase/homoserine dehydrogenase (AK/HSD) gene.hich encodes two linked key enzymes in the biosynthetic pathway of aspartate family amino acids has been studied . The conversion of aspartate into either the storage amino acid asparagine or aspartate family amino acids may be subject to a coordinated.eciprocal metabolic control.nd this biochemical branch point is a part of a larger.oordinated regulatory mechanism of nitrogen and carbon storage and utilization.
  IPR001048:Aspartate/glutamate/uridylate kinase
N -Acetylglutamate (NAG) fulfils distinct biological roles in lower and higher organisms. In prokaryotes.ower eukaryotes and plants it is the first intermediate in the biosynthesis of arginine.hereas in ureotelic (excreting nitrogen mostly in the form of urea) vertebrates.t is an essential allosteric cofactor for carbamyl phosphate synthetase I (CPSI).he first enzyme of the urea cycle. The pathway that leads from glutamate to arginine in lower organisms employs eight steps.tarting with the acetylation of glutamate to form NAG. In these species.AG can be produced by two enzymatic reactions: one catalysed by NAG synthase (NAGS) and the other by ornithine acetyltransferase (OAT). In ureotelic species.AG is produced exclusively by NAGS. In lower organisms.AGS is feedback-inhibited by L-arginine.hereas mammalian NAGS activity is significantly enhanced by this amino acid. The NAGS genes of bacteria.ungi and mammals are more diverse than other arginine-biosynthesis and urea-cycle genes. The evolutionary relationship between the distinctly different roles of NAG and its metabolism in lower and higher organisms remains to be determined .The pathway from glutamate to arginine is: NAGS; N-acetylglutamate synthase () (glutamate to N-acetylglutamate)NAGK; N-acetylglutamate kinase () (N-acetylglutamate to N-acetylglutamate-5P)NAGSA; N-acetyl-gamma-glutamyl-phosphate reductase () (N-acetylglutamate-5P to N-acetylglumate semialdehyde)Acetylornithine aminotransferase () (N-acetylglumate semialdehyde to N-acetylornithine)Acetylornithine deacetylase () (N-acetylornithine to ornithine)Arginase () (ornithine to arginine)This group represents a N-acetylglutamate synthase.nimal type .
  IPR011243:N-acetylglutamate synthase, animal
IPR006855:DUF619 
Evalue:-28.6020603179932 
Location:356-521
SequencesProtein: NAGS_HUMAN (534 aa)
mRNA: NM_153006
Local Annotation
Synapse Ontology
mitochondria are frequently observed in the vicinity of the synaptic vesicle clusters, in agreement with the ATP requirement of several steps of the vesicle cycle.
sdb:0118 mitochondria  (Evidence:keywords)
KO assignmentK00618
  Level 3 annotation:
    amino-acid N-acetyltransferase
  Level 2 annotation:
    Urea cycle and metabolism of amino groups
Loci Structure (Details)Loci index, Chromosomal location, Length, Possible relational loci clusterExon1: 143 residues, 39437557-39437983Exon2: 93 residues, 39438530-39438805Exon3: 73 residues, 39438917-39439131Exon4: 62 residues, 39439422-39439603Exon5: 59 residues, 39440216-39440388Exon6: 63 residues, 39440484-39440667Exon7: 208 residues, 39441341-39441961Exon8: 2 residues, -Jump to NAGS_HUMAN  
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Loci Cluster (Details)Loci: 4294 39509646-39556540 ~-47K 15975(HDAC5)(-)Loci: 4295 39682565-39700993 ~-18K 15992(SLC4A1)(-)Loci: 4296 39752520-39757666 ~-5K 15997(-)Loci: 3010 40098011-40104116 ~-6K 16007(+)Loci: 4297 40237112-40263147 ~-26K 16016(GJA7)(-)Loci: 4298 40338518-40348394 ~-10K 16022(GFAP)(-)Loci: 3009 39437557-39441961 ~-4K 15971(NAGS)(+)Link out to UCSC