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Protein

Glutamine synthetase

Gene

GLUL

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.By similarity1 Publication

Catalytic activityi

ATP + L-glutamate + NH3 = ADP + phosphate + L-glutamine.
L-glutamate = 4-aminobutanoate + CO2.

Cofactori

Protein has several cofactor binding sites:

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLigase, Lyase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS06066-MONOMER
BRENDAi6.3.1.2 2681
ReactomeiR-HSA-210455 Astrocytic Glutamate-Glutamine Uptake And Metabolism
R-HSA-70614 Amino acid synthesis and interconversion (transamination)
SABIO-RKiP15104

Names & Taxonomyi

Protein namesi
Recommended name:
Glutamine synthetase (EC:6.3.1.2)
Short name:
GS
Alternative name(s):
Glutamate decarboxylase (EC:4.1.1.15)
Glutamate--ammonia ligase
Gene namesi
Name:GLUL
Synonyms:GLNS
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000135821.16
HGNCiHGNC:4341 GLUL
MIMi138290 gene
neXtProtiNX_P15104

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

Cytoplasm, Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Congenital systemic glutamine deficiency (CSGD)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionRare developmental disorder with severe brain malformation resulting in multi-organ failure and neonatal death. Glutamine is largely absent from affected patients serum, urine and cerebrospinal fluid.
See also OMIM:610015
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_026560324R → C in CSGD; reduced glutamine synthetase activity. 1 PublicationCorresponds to variant dbSNP:rs80358214EnsemblClinVar.1
Natural variantiVAR_026561341R → C in CSGD; suggests reduced glutamine synthetase activity. 1 PublicationCorresponds to variant dbSNP:rs80358215EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi2752
MalaCardsiGLUL
MIMi610015 phenotype
OpenTargetsiENSG00000135821
Orphaneti71278 Congenital brain dysgenesis due to glutamine synthetase deficiency
PharmGKBiPA28743

Chemistry databases

ChEMBLiCHEMBL4612
DrugBankiDB06774 Capsaicin
DB01212 Ceftriaxone
DB01119 Diazoxide
DB08794 Ethyl biscoumacetate
DB00142 L-Glutamic Acid
DB00130 L-Glutamine
DB00134 L-Methionine
DB00082 Pegvisomant
DB00466 Picrotoxin

Polymorphism and mutation databases

BioMutaiGLUL

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00001531392 – 373Glutamine synthetaseAdd BLAST372

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylthreonineCombined sources1
Modified residuei104PhosphotyrosineBy similarity1
Modified residuei343PhosphoserineCombined sources1

Post-translational modificationi

Ubiquitinated by ZNRF1.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiP15104
MaxQBiP15104
PaxDbiP15104
PeptideAtlasiP15104
PRIDEiP15104
ProteomicsDBi53107

2D gel databases

REPRODUCTION-2DPAGEiIPI00010130
UCD-2DPAGEiP15104

PTM databases

iPTMnetiP15104
PhosphoSitePlusiP15104

Expressioni

Developmental stagei

Expressed during early fetal stages.1 Publication

Inductioni

By glucocorticoids. Vitamin D and the Wnt signaling pathway inhibit its expression and activity.1 Publication

Gene expression databases

BgeeiENSG00000135821 Expressed in 243 organ(s), highest expression level in medial globus pallidus
CleanExiHS_GLUL
ExpressionAtlasiP15104 baseline and differential
GenevisibleiP15104 HS

Organism-specific databases

HPAiCAB008636
HPA007316
HPA007571

Interactioni

Subunit structurei

Homooctamer and homotetramer. Interacts with PALMD (By similarity).By similarity

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi109014, 36 interactors
DIPiDIP-308N
IntActiP15104, 25 interactors
MINTiP15104
STRINGi9606.ENSP00000307900

Chemistry databases

BindingDBiP15104

Structurei

Secondary structure

1373
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP15104
SMRiP15104
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP15104

Family & Domainsi

Sequence similaritiesi

Belongs to the glutamine synthetase family.Curated

Phylogenomic databases

eggNOGiKOG0683 Eukaryota
COG0174 LUCA
GeneTreeiENSGT00390000010047
HOGENOMiHOG000061500
HOVERGENiHBG005847
InParanoidiP15104
KOiK01915
OMAiMMPDGVT
OrthoDBiEOG091G07E9
PhylomeDBiP15104
TreeFamiTF300491

Family and domain databases

Gene3Di3.10.20.70, 1 hit
InterProiView protein in InterPro
IPR008147 Gln_synt_b-grasp
IPR036651 Gln_synt_N
IPR014746 Gln_synth/guanido_kin_cat_dom
IPR008146 Gln_synth_cat_dom
IPR027303 Gln_synth_gly_rich_site
IPR027302 Gln_synth_N_conserv_site
PfamiView protein in Pfam
PF00120 Gln-synt_C, 1 hit
PF03951 Gln-synt_N, 1 hit
SMARTiView protein in SMART
SM01230 Gln-synt_C, 1 hit
SUPFAMiSSF54368 SSF54368, 1 hit
SSF55931 SSF55931, 1 hit
PROSITEiView protein in PROSITE
PS00180 GLNA_1, 1 hit
PS00181 GLNA_ATP, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.iShow all

P15104-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MTTSASSHLN KGIKQVYMSL PQGEKVQAMY IWIDGTGEGL RCKTRTLDSE
60 70 80 90 100
PKCVEELPEW NFDGSSTLQS EGSNSDMYLV PAAMFRDPFR KDPNKLVLCE
110 120 130 140 150
VFKYNRRPAE TNLRHTCKRI MDMVSNQHPW FGMEQEYTLM GTDGHPFGWP
160 170 180 190 200
SNGFPGPQGP YYCGVGADRA YGRDIVEAHY RACLYAGVKI AGTNAEVMPA
210 220 230 240 250
QWEFQIGPCE GISMGDHLWV ARFILHRVCE DFGVIATFDP KPIPGNWNGA
260 270 280 290 300
GCHTNFSTKA MREENGLKYI EEAIEKLSKR HQYHIRAYDP KGGLDNARRL
310 320 330 340 350
TGFHETSNIN DFSAGVANRS ASIRIPRTVG QEKKGYFEDR RPSANCDPFS
360 370
VTEALIRTCL LNETGDEPFQ YKN
Length:373
Mass (Da):42,064
Last modified:January 23, 2007 - v4
Checksum:i45390C100924FAF3
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A2R8YDT1A0A2R8YDT1_HUMAN
Glutamine synthetase
GLUL
507Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti7S → Y in AAH31964 (PubMed:15489334).Curated1
Sequence conflicti154F → L in CAD97626 (PubMed:17974005).Curated1
Sequence conflicti155P → T in AAH31964 (PubMed:15489334).Curated1
Sequence conflicti314A → G in CAA68457 (PubMed:2888076).Curated1
Sequence conflicti322 – 323SI → RL in CAA42495 (PubMed:1681907).Curated2
Sequence conflicti347D → E in CAA42495 (PubMed:1681907).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_026560324R → C in CSGD; reduced glutamine synthetase activity. 1 PublicationCorresponds to variant dbSNP:rs80358214EnsemblClinVar.1
Natural variantiVAR_026561341R → C in CSGD; suggests reduced glutamine synthetase activity. 1 PublicationCorresponds to variant dbSNP:rs80358215EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y00387 mRNA Translation: CAA68457.1
X59834 mRNA Translation: CAA42495.1
S70290 mRNA Translation: AAB30693.1
AY486122 mRNA Translation: AAS57904.1
AY486123 Genomic DNA Translation: AAS57905.1
BX537384 mRNA Translation: CAD97626.1
AL139344 Genomic DNA No translation available.
BC010037 mRNA Translation: AAH10037.1
BC011700 mRNA Translation: AAH11700.1
BC011852 mRNA Translation: AAH11852.1
BC018992 mRNA Translation: AAH18992.1
BC031964 mRNA Translation: AAH31964.1
BC051726 mRNA Translation: AAH51726.1
CCDSiCCDS1344.1
PIRiS18455 AJHUQ
RefSeqiNP_001028216.1, NM_001033044.3
NP_001028228.1, NM_001033056.3
NP_002056.2, NM_002065.6
XP_006711341.1, XM_006711278.1
UniGeneiHs.132016
Hs.518525

Genome annotation databases

EnsembliENST00000311223; ENSP00000307900; ENSG00000135821
ENST00000331872; ENSP00000356537; ENSG00000135821
ENST00000339526; ENSP00000344958; ENSG00000135821
ENST00000417584; ENSP00000398320; ENSG00000135821
GeneIDi2752
KEGGihsa:2752
UCSCiuc001gpa.3 human

Similar proteinsi

Cross-referencesi

Web resourcesi

Wikipedia

Glutamine synthetase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y00387 mRNA Translation: CAA68457.1
X59834 mRNA Translation: CAA42495.1
S70290 mRNA Translation: AAB30693.1
AY486122 mRNA Translation: AAS57904.1
AY486123 Genomic DNA Translation: AAS57905.1
BX537384 mRNA Translation: CAD97626.1
AL139344 Genomic DNA No translation available.
BC010037 mRNA Translation: AAH10037.1
BC011700 mRNA Translation: AAH11700.1
BC011852 mRNA Translation: AAH11852.1
BC018992 mRNA Translation: AAH18992.1
BC031964 mRNA Translation: AAH31964.1
BC051726 mRNA Translation: AAH51726.1
CCDSiCCDS1344.1
PIRiS18455 AJHUQ
RefSeqiNP_001028216.1, NM_001033044.3
NP_001028228.1, NM_001033056.3
NP_002056.2, NM_002065.6
XP_006711341.1, XM_006711278.1
UniGeneiHs.132016
Hs.518525

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2OJWX-ray2.05A/B/C/D/E5-365[»]
2QC8X-ray2.60A/B/C/D/E/F/G/H/I/J5-365[»]
ProteinModelPortaliP15104
SMRiP15104
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109014, 36 interactors
DIPiDIP-308N
IntActiP15104, 25 interactors
MINTiP15104
STRINGi9606.ENSP00000307900

Chemistry databases

BindingDBiP15104
ChEMBLiCHEMBL4612
DrugBankiDB06774 Capsaicin
DB01212 Ceftriaxone
DB01119 Diazoxide
DB08794 Ethyl biscoumacetate
DB00142 L-Glutamic Acid
DB00130 L-Glutamine
DB00134 L-Methionine
DB00082 Pegvisomant
DB00466 Picrotoxin

PTM databases

iPTMnetiP15104
PhosphoSitePlusiP15104

Polymorphism and mutation databases

BioMutaiGLUL

2D gel databases

REPRODUCTION-2DPAGEiIPI00010130
UCD-2DPAGEiP15104

Proteomic databases

EPDiP15104
MaxQBiP15104
PaxDbiP15104
PeptideAtlasiP15104
PRIDEiP15104
ProteomicsDBi53107

Protocols and materials databases

DNASUi2752
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000311223; ENSP00000307900; ENSG00000135821
ENST00000331872; ENSP00000356537; ENSG00000135821
ENST00000339526; ENSP00000344958; ENSG00000135821
ENST00000417584; ENSP00000398320; ENSG00000135821
GeneIDi2752
KEGGihsa:2752
UCSCiuc001gpa.3 human

Organism-specific databases

CTDi2752
DisGeNETi2752
EuPathDBiHostDB:ENSG00000135821.16
GeneCardsiGLUL
HGNCiHGNC:4341 GLUL
HPAiCAB008636
HPA007316
HPA007571
MalaCardsiGLUL
MIMi138290 gene
610015 phenotype
neXtProtiNX_P15104
OpenTargetsiENSG00000135821
Orphaneti71278 Congenital brain dysgenesis due to glutamine synthetase deficiency
PharmGKBiPA28743
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0683 Eukaryota
COG0174 LUCA
GeneTreeiENSGT00390000010047
HOGENOMiHOG000061500
HOVERGENiHBG005847
InParanoidiP15104
KOiK01915
OMAiMMPDGVT
OrthoDBiEOG091G07E9
PhylomeDBiP15104
TreeFamiTF300491

Enzyme and pathway databases

BioCyciMetaCyc:HS06066-MONOMER
BRENDAi6.3.1.2 2681
ReactomeiR-HSA-210455 Astrocytic Glutamate-Glutamine Uptake And Metabolism
R-HSA-70614 Amino acid synthesis and interconversion (transamination)
SABIO-RKiP15104

Miscellaneous databases

ChiTaRSiGLUL human
EvolutionaryTraceiP15104
GenomeRNAii2752
PROiPR:P15104
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000135821 Expressed in 243 organ(s), highest expression level in medial globus pallidus
CleanExiHS_GLUL
ExpressionAtlasiP15104 baseline and differential
GenevisibleiP15104 HS

Family and domain databases

Gene3Di3.10.20.70, 1 hit
InterProiView protein in InterPro
IPR008147 Gln_synt_b-grasp
IPR036651 Gln_synt_N
IPR014746 Gln_synth/guanido_kin_cat_dom
IPR008146 Gln_synth_cat_dom
IPR027303 Gln_synth_gly_rich_site
IPR027302 Gln_synth_N_conserv_site
PfamiView protein in Pfam
PF00120 Gln-synt_C, 1 hit
PF03951 Gln-synt_N, 1 hit
SMARTiView protein in SMART
SM01230 Gln-synt_C, 1 hit
SUPFAMiSSF54368 SSF54368, 1 hit
SSF55931 SSF55931, 1 hit
PROSITEiView protein in PROSITE
PS00180 GLNA_1, 1 hit
PS00181 GLNA_ATP, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiGLNA_HUMAN
AccessioniPrimary (citable) accession number: P15104
Secondary accession number(s): Q499Y9
, Q5T9Z1, Q7Z3W4, Q8IZ17
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1990
Last sequence update: January 23, 2007
Last modified: September 12, 2018
This is version 201 of the entry and version 4 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome
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Main funding by: National Institutes of Health

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