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Protein

N-acetylglutamate synthase, mitochondrial

Gene

Nags

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Plays a role in the regulation of ureagenesis by producing the essential cofactor N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.

Catalytic activityi

Acetyl-CoA + L-glutamate = CoA + N-acetyl-L-glutamate.

Enzyme regulationi

Increased by L-arginine.1 Publication

Pathwayi: L-arginine biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes N(2)-acetyl-L-ornithine from L-glutamate.
Proteins known to be involved in the 4 steps of the subpathway in this organism are:
  1. N-acetylglutamate synthase, mitochondrial (Nags)
  2. no protein annotated in this organism
  3. no protein annotated in this organism
  4. no protein annotated in this organism
This subpathway is part of the pathway L-arginine biosynthesis, which is itself part of Amino-acid biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes N(2)-acetyl-L-ornithine from L-glutamate, the pathway L-arginine biosynthesis and in Amino-acid biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei394SubstrateBy similarity1
Binding sitei437SubstrateBy similarity1

GO - Molecular functioni

GO - Biological processi

  • arginine biosynthetic process Source: GO_Central
  • glutamate metabolic process Source: MGI
  • urea cycle Source: UniProtKB-KW

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processUrea cycle

Enzyme and pathway databases

BRENDAi2.3.1.1 3474
ReactomeiR-MMU-70635 Urea cycle
UniPathwayiUPA00068; UER00106

Names & Taxonomyi

Protein namesi
Recommended name:
N-acetylglutamate synthase, mitochondrial (EC:2.3.1.1)
Alternative name(s):
Amino-acid acetyltransferase
Cleaved into the following 3 chains:
Gene namesi
Name:Nags
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi

Organism-specific databases

MGIiMGI:2387600 Nags

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 18MitochondrionSequence analysisAdd BLAST18
ChainiPRO_000004193319 – 527N-acetylglutamate synthase long formSequence analysisAdd BLAST509
ChainiPRO_000004193451 – 527N-acetylglutamate synthase short formAdd BLAST477
ChainiPRO_000004193584 – 527N-acetylglutamate synthase conserved domain formAdd BLAST444

Post-translational modificationi

Probably processed by mitochondrial processing peptidase (MPP). The long form has not yet been isolated.1 Publication

Proteomic databases

PaxDbiQ8R4H7
PeptideAtlasiQ8R4H7
PRIDEiQ8R4H7

PTM databases

iPTMnetiQ8R4H7
PhosphoSitePlusiQ8R4H7
SwissPalmiQ8R4H7

Expressioni

Tissue specificityi

Highly expressed in the liver and small intestine. Weakly expressed in the kidney, spleen and testis.1 Publication

Gene expression databases

BgeeiENSMUSG00000048217
CleanExiMM_NAGS
GenevisibleiQ8R4H7 MM

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000050258

Structurei

3D structure databases

ProteinModelPortaliQ8R4H7
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini368 – 519N-acetyltransferasePROSITE-ProRule annotationAdd BLAST152

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni19 – 370Amino-acid kinase domain (AAK)By similarityAdd BLAST352
Regioni467 – 472Substrate bindingBy similarity6

Domaini

The Amino-acid kinase domain (AAK) mediates binding of the allosteric activator L-arginine.By similarity

Sequence similaritiesi

Belongs to the acetyltransferase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2436 Eukaryota
COG0548 LUCA
GeneTreeiENSGT00390000005602
HOGENOMiHOG000007983
HOVERGENiHBG080036
InParanoidiQ8R4H7
KOiK11067
OMAiGSIPILC
OrthoDBiEOG091G0FPD
PhylomeDBiQ8R4H7
TreeFamiTF332628

Family and domain databases

Gene3Di3.40.1160.10, 1 hit
InterProiView protein in InterPro
IPR036393 AceGlu_kinase-like_sf
IPR016181 Acyl_CoA_acyltransferase
IPR011243 GlcNAc_Synth_met
IPR006855 Vertebrate-like_GNAT_dom
PfamiView protein in Pfam
PF04768 NAT, 1 hit
PIRSFiPIRSF036442 NAGS_animal, 1 hit
SUPFAMiSSF53633 SSF53633, 1 hit
SSF55729 SSF55729, 1 hit
PROSITEiView protein in PROSITE
PS51731 GNAT_NAGS, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8R4H7-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MATAWVATAL RSAAAARRLR SPGGPGGSRR LSGSARRRGA KSASPGRRLS
60 70 80 90 100
TARAHAEDAE GAKGRVQSPA VEEPSWTPLP TPLESPAPPA GRSLVQRDIQ
110 120 130 140 150
AFLNQCGASP GEARHWLTQF QTCYHSVDKP FAVMEVDEEV IRCPQAVSRL
160 170 180 190 200
AFALAFLQRM DMKPLVVLGL PTPTAPSGCL SFWEAKAQLA QSCKVLVDEL
210 220 230 240 250
RHNAATAVPF FGGGSVLSAA EPAPHASYGG IVAVETDLLQ WCLESNSIPI
260 270 280 290 300
LCPIGETAAR RSVLLDSLEV TASLAKALQP TKIIFLNNSG GLRNNSQKIL
310 320 330 340 350
SNVNLPADLD LVTNAEWLSI KERQQIRLIV DVLSRLPHYS SAVITAASTL
360 370 380 390 400
LTELFSNKGC GTLFKNAERM LRVRNLDSLD QGRLVNLVNA SFGKKLREDY
410 420 430 440 450
LESLRPRLHS IYVSEGYNAA AILTVEPVLG GTPYLDKFVV SSSRQGQGSG
460 470 480 490 500
QMLWECLRRD LQTLFWRSRV TNPINPWYFK HSDGSFSNKQ WIFFWFGLAD
510 520
IRDSYELVNH AKGLPDSFCK PASDPGS
Length:527
Mass (Da):57,489
Last modified:September 13, 2005 - v2
Checksum:i3A39E92955B11231
GO
Isoform 2 (identifier: Q8R4H7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-159: Missing.

Note: No experimental confirmation available.
Show »
Length:368
Mass (Da):40,480
Checksum:i43CD6AD1649191D6
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti72 – 75EEPS → DPRVR in AAL86770 (PubMed:12049647).Curated4

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0156201 – 159Missing in isoform 2. 1 PublicationAdd BLAST159

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF462069 mRNA Translation: AAL86770.1
AJ489814 mRNA Translation: CAD34015.1
AK075765 mRNA Translation: BAC35941.1
AL591145 Genomic DNA Translation: CAM23258.1
CH466558 Genomic DNA Translation: EDL34091.1
BC057990 mRNA Translation: AAH57990.1
CCDSiCCDS25488.1 [Q8R4H7-1]
RefSeqiNP_665828.1, NM_145829.2 [Q8R4H7-1]
UniGeneiMm.31686

Genome annotation databases

EnsembliENSMUST00000055409; ENSMUSP00000050258; ENSMUSG00000048217 [Q8R4H7-1]
GeneIDi217214
KEGGimmu:217214
UCSCiuc007lqp.2 mouse [Q8R4H7-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiNAGS_MOUSE
AccessioniPrimary (citable) accession number: Q8R4H7
Secondary accession number(s): B1AQG2
, Q8C6G6, Q8CI77, Q8K1R8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 13, 2005
Last sequence update: September 13, 2005
Last modified: May 23, 2018
This is version 123 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

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