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Protein

Glutamine--tRNA ligase

Gene

QARS

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

Functioni

Glutamine--tRNA ligase (PubMed:26869582). Plays a critical role in brain development (PubMed:24656866).2 Publications

Catalytic activityi

ATP + L-glutamine + tRNA(Gln) = AMP + diphosphate + L-glutaminyl-tRNA(Gln).1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei303L-glutamineBy similarity1
Binding sitei438L-glutamineBy similarity1
Binding sitei457ATPBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi271 – 273ATPBy similarity3
Nucleotide bindingi277 – 283ATPBy similarity7
Nucleotide bindingi486 – 487ATPBy similarity2
Nucleotide bindingi494 – 496ATPBy similarity3

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • glutamine-tRNA ligase activity Source: UniProtKB
  • protein kinase binding Source: CAFA
  • protein kinase inhibitor activity Source: CAFA

GO - Biological processi

  • brain development Source: UniProtKB
  • glutaminyl-tRNA aminoacylation Source: UniProtKB
  • negative regulation of apoptotic signaling pathway Source: CAFA
  • negative regulation of protein kinase activity Source: CAFA
  • negative regulation of stress-activated MAPK cascade Source: CAFA
  • negative regulation of transcription, DNA-templated Source: CAFA
  • tRNA aminoacylation for protein translation Source: Reactome

Keywordsi

Molecular functionAminoacyl-tRNA synthetase, Ligase
Biological processProtein biosynthesis
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-HSA-2408517 SeMet incorporation into proteins
R-HSA-379716 Cytosolic tRNA aminoacylation
R-HSA-379726 Mitochondrial tRNA aminoacylation

Protein family/group databases

MoonProtiP47897

Names & Taxonomyi

Protein namesi
Recommended name:
Glutamine--tRNA ligase (EC:6.1.1.182 Publications)
Alternative name(s):
Glutaminyl-tRNA synthetase
Short name:
GlnRS2 Publications
Gene namesi
Name:QARS
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000172053.15
HGNCiHGNC:9751 QARS
MIMi603727 gene
neXtProtiNX_P47897

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

Pathology & Biotechi

Involvement in diseasei

Microcephaly, progressive, with seizures and cerebral and cerebellar atrophy (MSCCA)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA severe, autosomal recessive, neurodevelopmental and neurodegenerative disorder characterized by progressive microcephaly, severe seizures in infancy, atrophy of the cerebral cortex and cerebellar vermis, and mild atrophy of the cerebellar hemispheres, resulting in profoundly delayed development and hypotonia.
See also OMIM:615760
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07118945G → V in MSCCA; results in reduced glutaminyl-tRNA aminoacylation activity; does not affect interaction with RARS. 2 PublicationsCorresponds to variant dbSNP:rs587777331EnsemblClinVar.1
Natural variantiVAR_07119057Y → H in MSCCA; results in reduced glutaminyl-tRNA aminoacylation activity; does not affect interaction with RARS. 2 PublicationsCorresponds to variant dbSNP:rs587777333EnsemblClinVar.1
Natural variantiVAR_071191403R → W in MSCCA; results in loss of glutaminyl-tRNA aminoacylation activity; impairs protein folding; does not interact with RARS; results in reduced protein solubility. 2 PublicationsCorresponds to variant dbSNP:rs587777332EnsemblClinVar.1
Natural variantiVAR_071192515R → W in MSCCA; results in loss of glutaminyl-tRNA aminoacylation activity; impairs protein folding; results in reduced protein solubility. 2 PublicationsCorresponds to variant dbSNP:rs587777334EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi175H → A: Decreases catalytic efficiency about 60-fold. 1 Publication1

Keywords - Diseasei

Disease mutation, Epilepsy, Primary microcephaly

Organism-specific databases

DisGeNETi5859
MalaCardsiQARS
MIMi615760 phenotype
OpenTargetsiENSG00000172053
Orphaneti404437 Diffuse cerebral and cerebellar atrophy-intractable seizures-progressive microcephaly syndrome
PharmGKBiPA34093

Chemistry databases

ChEMBLiCHEMBL3054
DrugBankiDB00130 L-Glutamine

Polymorphism and mutation databases

DMDMi1351170

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00001958602 – 775Glutamine--tRNA ligaseAdd BLAST774

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1 Publication1
Modified residuei70PhosphoserineCombined sources1
Modified residuei309N6-acetyllysineCombined sources1
Modified residuei495PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP47897
MaxQBiP47897
PaxDbiP47897
PeptideAtlasiP47897
PRIDEiP47897
ProteomicsDBi55816

PTM databases

iPTMnetiP47897
PhosphoSitePlusiP47897
SwissPalmiP47897

Expressioni

Tissue specificityi

Highly expressed in fetal cerebral cortex, particularly in the ventricular zone, inner subventricular zone, outer subventricular zone, and cortical plate.1 Publication

Gene expression databases

BgeeiENSG00000172053
CleanExiHS_QARS
ExpressionAtlasiP47897 baseline and differential
GenevisibleiP47897 HS

Organism-specific databases

HPAiHPA036986
HPA036987

Interactioni

Subunit structurei

Monomer (PubMed:26869582). Part of a multisubunit complex that groups tRNA ligases for Arg (RARS), Asp (DARS), Gln (QARS), Ile (IARS), Leu (LARS), Lys (KARS), Met (MARS) the bifunctional ligase for Glu and Pro (EPRS) and the auxiliary subunits AIMP1/p43, AIMP2/p38 and EEF1E1/p18 (PubMed:19131329, PubMed:19289464). Interacts with RARS (PubMed:24656866). Part of a complex composed of RARS, QARS and AIMP1 (PubMed:25288775).5 Publications

Binary interactionsi

Show more details

GO - Molecular functioni

  • protein kinase binding Source: CAFA

Protein-protein interaction databases

BioGridi111797, 195 interactors
CORUMiP47897
IntActiP47897, 114 interactors
MINTiP47897
STRINGi9606.ENSP00000307567

Chemistry databases

BindingDBiP47897

Structurei

Secondary structure

1775
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi6 – 12Combined sources7
Helixi17 – 25Combined sources9
Helixi27 – 42Combined sources16
Turni43 – 45Combined sources3
Helixi50 – 62Combined sources13
Helixi66 – 68Combined sources3
Helixi69 – 77Combined sources9
Helixi84 – 94Combined sources11
Helixi104 – 110Combined sources7
Helixi119 – 140Combined sources22
Helixi141 – 143Combined sources3
Helixi147 – 154Combined sources8
Helixi162 – 177Combined sources16
Helixi220 – 223Combined sources4
Helixi226 – 229Combined sources4
Helixi237 – 239Combined sources3
Helixi249 – 260Combined sources12
Beta strandi265 – 268Combined sources4
Helixi278 – 293Combined sources16
Beta strandi297 – 302Combined sources6
Helixi312 – 324Combined sources13
Beta strandi330 – 334Combined sources5
Helixi335 – 338Combined sources4
Helixi339 – 351Combined sources13
Beta strandi354 – 358Combined sources5
Turni374 – 377Combined sources4
Helixi380 – 391Combined sources12
Beta strandi401 – 404Combined sources4
Beta strandi416 – 420Combined sources5
Turni426 – 428Combined sources3
Beta strandi433 – 436Combined sources4
Helixi438 – 448Combined sources11
Beta strandi452 – 457Combined sources6
Helixi464 – 473Combined sources10
Beta strandi480 – 484Combined sources5
Beta strandi487 – 489Combined sources3
Helixi496 – 504Combined sources9
Beta strandi507 – 510Combined sources4
Helixi519 – 524Combined sources6
Helixi529 – 539Combined sources11
Beta strandi546 – 548Combined sources3
Helixi551 – 564Combined sources14
Beta strandi570 – 576Combined sources7
Beta strandi578 – 581Combined sources4
Beta strandi592 – 594Combined sources3
Helixi599 – 601Combined sources3
Beta strandi603 – 606Combined sources4
Beta strandi613 – 615Combined sources3
Turni616 – 618Combined sources3
Beta strandi647 – 650Combined sources4
Beta strandi654 – 656Combined sources3
Beta strandi661 – 663Combined sources3
Beta strandi681 – 683Combined sources3
Beta strandi685 – 692Combined sources8
Beta strandi695 – 700Combined sources6
Turni702 – 705Combined sources4
Turni711 – 714Combined sources4
Beta strandi720 – 722Combined sources3
Beta strandi725 – 728Combined sources4
Beta strandi732 – 734Combined sources3
Beta strandi740 – 743Combined sources4
Turni744 – 746Combined sources3
Beta strandi747 – 751Combined sources5
Beta strandi757 – 759Combined sources3
Beta strandi761 – 766Combined sources6

3D structure databases

ProteinModelPortaliP47897
SMRiP47897
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi270 – 280"HIGH" region1 PublicationAdd BLAST11
Motifi493 – 497"KMSKS" region1 Publication5

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG1148 Eukaryota
COG0008 LUCA
GeneTreeiENSGT00550000074972
HOGENOMiHOG000259233
HOVERGENiHBG000889
InParanoidiP47897
KOiK01886
OMAiVTHSICT
OrthoDBiEOG091G026F
PhylomeDBiP47897
TreeFamiTF105683

Family and domain databases

Gene3Di2.40.240.10, 3 hits
3.40.50.620, 1 hit
InterProiView protein in InterPro
IPR001412 aa-tRNA-synth_I_CS
IPR004514 Gln-tRNA-synth
IPR007638 Gln-tRNA-synth_Ib_RNA-bd_2
IPR007639 Gln-tRNA-synth_Ib_RNA-bd_N
IPR000924 Glu/Gln-tRNA-synth
IPR020058 Glu/Gln-tRNA-synth_Ib_cat-dom
IPR020059 Glu/Gln-tRNA-synth_Ib_codon-bd
IPR020056 Rbsml_L25/Gln-tRNA_synth_N
IPR011035 Ribosomal_L25/Gln-tRNA_synth
IPR014729 Rossmann-like_a/b/a_fold
PfamiView protein in Pfam
PF00749 tRNA-synt_1c, 1 hit
PF03950 tRNA-synt_1c_C, 1 hit
PF04558 tRNA_synt_1c_R1, 1 hit
PF04557 tRNA_synt_1c_R2, 1 hit
PRINTSiPR00987 TRNASYNTHGLU
SUPFAMiSSF50715 SSF50715, 1 hit
TIGRFAMsiTIGR00440 glnS, 1 hit
PROSITEiView protein in PROSITE
PS00178 AA_TRNA_LIGASE_I, 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: P47897-1) [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
MAALDSLSLF TSLGLSEQKA RETLKNSALS AQLREAATQA QQTLGSTIDK
60 70 80 90 100
ATGILLYGLA SRLRDTRRLS FLVSYIASKK IHTEPQLSAA LEYVRSHPLD
110 120 130 140 150
PIDTVDFERE CGVGVIVTPE QIEEAVEAAI NRHRPQLLVE RYHFNMGLLM
160 170 180 190 200
GEARAVLKWA DGKMIKNEVD MQVLHLLGPK LEADLEKKFK VAKARLEETD
210 220 230 240 250
RRTAKDVVEN GETADQTLSL MEQLRGEALK FHKPGENYKT PGYVVTPHTM
260 270 280 290 300
NLLKQHLEIT GGQVRTRFPP EPNGILHIGH AKAINFNFGY AKANNGICFL
310 320 330 340 350
RFDDTNPEKE EAKFFTAICD MVAWLGYTPY KVTYASDYFD QLYAWAVELI
360 370 380 390 400
RRGLAYVCHQ RGEELKGHNT LPSPWRDRPM EESLLLFEAM RKGKFSEGEA
410 420 430 440 450
TLRMKLVMED GKMDPVAYRV KYTPHHRTGD KWCIYPTYDY THCLCDSIEH
460 470 480 490 500
ITHSLCTKEF QARRSSYFWL CNALDVYCPV QWEYGRLNLH YAVVSKRKIL
510 520 530 540 550
QLVATGAVRD WDDPRLFTLT ALRRRGFPPE AINNFCARVG VTVAQTTMEP
560 570 580 590 600
HLLEACVRDV LNDTAPRAMA VLESLRVIIT NFPAAKSLDI QVPNFPADET
610 620 630 640 650
KGFHQVPFAP IVFIERTDFK EEPEPGFKRL AWGQPVGLRH TGYVIELQHV
660 670 680 690 700
VKGPSGCVES LEVTCRRADA GEKPKAFIHW VSQPLMCEVR LYERLFQHKN
710 720 730 740 750
PEDPTEVPGG FLSDLNLASL HVVDAALVDC SVALAKPFDK FQFERLGYFS
760 770
VDPDSHQGKL VFNRTVTLKE DPGKV
Length:775
Mass (Da):87,799
Last modified:February 1, 1996 - v1
Checksum:iADDE23E6C442FF73
GO
Isoform 2 (identifier: P47897-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     78-89: SKKIHTEPQLSA → T

Show »
Length:764
Mass (Da):86,579
Checksum:iBA9B1C92EE0C2F72
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07118945G → V in MSCCA; results in reduced glutaminyl-tRNA aminoacylation activity; does not affect interaction with RARS. 2 PublicationsCorresponds to variant dbSNP:rs587777331EnsemblClinVar.1
Natural variantiVAR_07119057Y → H in MSCCA; results in reduced glutaminyl-tRNA aminoacylation activity; does not affect interaction with RARS. 2 PublicationsCorresponds to variant dbSNP:rs587777333EnsemblClinVar.1
Natural variantiVAR_071191403R → W in MSCCA; results in loss of glutaminyl-tRNA aminoacylation activity; impairs protein folding; does not interact with RARS; results in reduced protein solubility. 2 PublicationsCorresponds to variant dbSNP:rs587777332EnsemblClinVar.1
Natural variantiVAR_071192515R → W in MSCCA; results in loss of glutaminyl-tRNA aminoacylation activity; impairs protein folding; results in reduced protein solubility. 2 PublicationsCorresponds to variant dbSNP:rs587777334EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_05510778 – 89SKKIH…PQLSA → T in isoform 2. 1 PublicationAdd BLAST12

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X76013 mRNA Translation: CAA53600.1
AK301559 mRNA Translation: BAG63054.1
AC135506 Genomic DNA No translation available.
BC000394 mRNA Translation: AAH00394.1
BC001567 mRNA Translation: AAH01567.1
BC029739 mRNA Translation: AAH29739.1
CCDSiCCDS2788.1 [P47897-1]
CCDS63633.1 [P47897-2]
PIRiI37422
RefSeqiNP_001259002.1, NM_001272073.1 [P47897-2]
NP_005042.1, NM_005051.2 [P47897-1]
UniGeneiHs.79322

Genome annotation databases

EnsembliENST00000306125; ENSP00000307567; ENSG00000172053 [P47897-1]
ENST00000414533; ENSP00000390015; ENSG00000172053 [P47897-2]
GeneIDi5859
KEGGihsa:5859
UCSCiuc003cvx.5 human [P47897-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiSYQ_HUMAN
AccessioniPrimary (citable) accession number: P47897
Secondary accession number(s): B4DWJ2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: February 1, 1996
Last modified: July 18, 2018
This is version 185 of the entry and version 1 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, Direct protein sequencing, Reference proteome

Documents

  1. Aminoacyl-tRNA synthetases
    List of aminoacyl-tRNA synthetase entries
  2. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

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