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Protein

Threonine--tRNA ligase, mitochondrial

Gene

TARS2

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

Functioni

Catalyzes the attachment of threonine to tRNA(Thr) in a two-step reaction: threonine is first activated by ATP to form Thr-AMP and then transferred to the acceptor end of tRNA(Thr). Also edits incorrectly charged tRNA(Thr) via its editing domain.1 Publication

Catalytic activityi

ATP + L-threonine + tRNA(Thr) = AMP + diphosphate + L-threonyl-tRNA(Thr).1 Publication

Kineticsi

kcat is 5.8 sec(-1) for the activation reaction of L-threonine. kcat is 1.1 sec(-1) for the activation reaction of L-serine. kcat is 0.061 sec(-1) for the aminoacylation of tRNA(Thr) with L-threonine.1 Publication
  1. KM=2.5 mM for L-threonine1 Publication
  2. KM=1.1 µM for tRNA(Thr)1 Publication
  3. KM=180 mM for L-serine1 Publication

    GO - Molecular functioni

    • aminoacyl-tRNA editing activity Source: UniProtKB
    • ATP binding Source: UniProtKB-KW
    • protein homodimerization activity Source: UniProtKB
    • threonine-tRNA ligase activity Source: UniProtKB

    GO - Biological processi

    • mitochondrial threonyl-tRNA aminoacylation Source: BHF-UCL

    Keywordsi

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

    Enzyme and pathway databases

    ReactomeiR-HSA-379726 Mitochondrial tRNA aminoacylation

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Threonine--tRNA ligase, mitochondrial (EC:6.1.1.31 Publication)
    Alternative name(s):
    Threonyl-tRNA synthetase
    Short name:
    ThrRS
    Threonyl-tRNA synthetase-like 1
    Gene namesi
    Name:TARS2
    Synonyms:TARSL1
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    Proteomesi
    • UP000005640 Componenti: Chromosome 1

    Organism-specific databases

    EuPathDBiHostDB:ENSG00000143374.14
    HGNCiHGNC:30740 TARS2
    MIMi612805 gene
    neXtProtiNX_Q9BW92

    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

    Pathology & Biotechi

    Involvement in diseasei

    Combined oxidative phosphorylation deficiency 21 (COXPD21)2 Publications
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionA mitochondrial disorder characterized by a lethal encephalomyopathy. Shortly after birth, affected individuals manifest axial hypotonia, limb hypertonia, psychomotor delay, and increased serum lactate. Additional features include subsarcolemmal lipofuscin-positive deposits in muscle, cerebral spongiosis, and hepatic steatosis.
    See also OMIM:615918
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_071853282P → L in COXPD21; decreased expression at mRNA and protein levels; decreased threonine-tRNA ligase activity; affects both Thr activation and transfer; decreased aminoacyl-tRNA editing activity; decreased protein stability; loss of homodimerization. 2 PublicationsCorresponds to variant dbSNP:rs587777593EnsemblClinVar.1

    Keywords - Diseasei

    Disease mutation, Primary mitochondrial disease

    Organism-specific databases

    DisGeNETi80222
    MalaCardsiTARS2
    MIMi615918 phenotype
    OpenTargetsiENSG00000143374
    PharmGKBiPA162405200

    Chemistry databases

    ChEMBLiCHEMBL3351186
    DrugBankiDB00156 L-Threonine

    Polymorphism and mutation databases

    DMDMi74752395

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_0000254586? – 718Threonine--tRNA ligase, mitochondrial
    Transit peptidei1 – ?MitochondrionSequence analysis

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei52PhosphoserineCombined sources1

    Keywords - PTMi

    Phosphoprotein

    Proteomic databases

    EPDiQ9BW92
    MaxQBiQ9BW92
    PaxDbiQ9BW92
    PeptideAtlasiQ9BW92
    PRIDEiQ9BW92
    ProteomicsDBi79266

    PTM databases

    iPTMnetiQ9BW92
    PhosphoSitePlusiQ9BW92

    Expressioni

    Gene expression databases

    BgeeiENSG00000143374
    CleanExiHS_TARS2
    ExpressionAtlasiQ9BW92 baseline and differential
    GenevisibleiQ9BW92 HS

    Organism-specific databases

    HPAiHPA028626

    Interactioni

    Subunit structurei

    Homodimer.1 Publication

    Binary interactionsi

    WithEntry#Exp.IntActNotes
    TARSL2A2RTX54EBI-1045099,EBI-1056629

    GO - Molecular functioni

    • protein homodimerization activity Source: UniProtKB

    Protein-protein interaction databases

    BioGridi123188, 13 interactors
    IntActiQ9BW92, 16 interactors
    MINTiQ9BW92
    STRINGi9606.ENSP00000358060

    Structurei

    3D structure databases

    ProteinModelPortaliQ9BW92
    SMRiQ9BW92
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiKOG1637 Eukaryota
    COG0441 LUCA
    GeneTreeiENSGT00390000002149
    HOGENOMiHOG000003878
    HOVERGENiHBG059513
    InParanoidiQ9BW92
    KOiK01868
    OMAiFIRAEYA
    OrthoDBiEOG091G01UL
    PhylomeDBiQ9BW92
    TreeFamiTF300858

    Family and domain databases

    CDDicd00771 ThrRS_core, 1 hit
    Gene3Di3.10.20.30, 1 hit
    3.40.50.800, 1 hit
    HAMAPiMF_00184 Thr_tRNA_synth, 1 hit
    InterProiView protein in InterPro
    IPR002314 aa-tRNA-synt_IIb
    IPR006195 aa-tRNA-synth_II
    IPR004154 Anticodon-bd
    IPR036621 Anticodon-bd_dom_sf
    IPR012675 Beta-grasp_dom_sf
    IPR004095 TGS
    IPR012676 TGS-like
    IPR002320 Thr-tRNA-ligase_IIa
    IPR018163 Thr/Ala-tRNA-synth_IIc_edit
    IPR033728 ThrRS_core
    IPR012947 tRNA_SAD
    PANTHERiPTHR11451 PTHR11451, 1 hit
    PfamiView protein in Pfam
    PF03129 HGTP_anticodon, 1 hit
    PF02824 TGS, 1 hit
    PF00587 tRNA-synt_2b, 1 hit
    PF07973 tRNA_SAD, 1 hit
    PRINTSiPR01047 TRNASYNTHTHR
    SMARTiView protein in SMART
    SM00863 tRNA_SAD, 1 hit
    SUPFAMiSSF55186 SSF55186, 1 hit
    SSF81271 SSF81271, 1 hit
    TIGRFAMsiTIGR00418 thrS, 1 hit
    PROSITEiView protein in PROSITE
    PS50862 AA_TRNA_LIGASE_II, 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: Q9BW92-1) [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
    MALYQRWRCL RLQGLQACRL HTAVVSTPPR WLAERLGLFE ELWAAQVKRL
    60 70 80 90 100
    ASMAQKEPRT IKISLPGGQK IDAVAWNTTP YQLARQISST LADTAVAAQV
    110 120 130 140 150
    NGEPYDLERP LETDSDLRFL TFDSPEGKAV FWHSSTHVLG AAAEQFLGAV
    160 170 180 190 200
    LCRGPSTEYG FYHDFFLGKE RTIRGSELPV LERICQELTA AARPFRRLEA
    210 220 230 240 250
    SRDQLRQLFK DNPFKLHLIE EKVTGPTATV YGCGTLVDLC QGPHLRHTGQ
    260 270 280 290 300
    IGGLKLLSNS SSLWRSSGAP ETLQRVSGIS FPTTELLRVW EAWREEAELR
    310 320 330 340 350
    DHRRIGKEQE LFFFHELSPG SCFFLPRGTR VYNALVAFIR AEYAHRGFSE
    360 370 380 390 400
    VKTPTLFSTK LWEQSGHWEH YQEDMFAVQP PGSDRPPSSQ SDDSTRHITD
    410 420 430 440 450
    TLALKPMNCP AHCLMFAHRP RSWRELPLRL ADFGALHRAE ASGGLGGLTR
    460 470 480 490 500
    LRCFQQDDAH IFCTTDQLEA EIQSCLDFLR SVYAVLGFSF RLALSTRPSG
    510 520 530 540 550
    FLGDPCLWDQ AEQVLKQALK EFGEPWDLNS GDGAFYGPKI DVHLHDALGR
    560 570 580 590 600
    PHQCGTIQLD FQLPLRFDLQ YKGQAGALER PVLIHRAVLG SVERLLGVLA
    610 620 630 640 650
    ESCGGKWPLW LSPFQVVVIP VGSEQEEYAK EAQQSLRAAG LVSDLDADSG
    660 670 680 690 700
    LTLSRRIRRA QLAHYNFQFV VGQKEQSKRT VNIRTRDNRR LGEWDLPEAV
    710
    QRLVELQNTR VPNAEEIF
    Length:718
    Mass (Da):81,036
    Last modified:June 1, 2001 - v1
    Checksum:iA2F793A60483E7F9
    GO
    Isoform 2 (identifier: Q9BW92-2) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         211-340: Missing.

    Note: No experimental confirmation available.
    Show »
    Length:588
    Mass (Da):66,372
    Checksum:i19A2BD2A6BB8C400
    GO

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Sequence conflicti269A → P in BAD96534 (Ref. 2) Curated1
    Sequence conflicti579E → G in BAD96534 (Ref. 2) Curated1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_071853282P → L in COXPD21; decreased expression at mRNA and protein levels; decreased threonine-tRNA ligase activity; affects both Thr activation and transfer; decreased aminoacyl-tRNA editing activity; decreased protein stability; loss of homodimerization. 2 PublicationsCorresponds to variant dbSNP:rs587777593EnsemblClinVar.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Alternative sequenceiVSP_054537211 – 340Missing in isoform 2. 1 PublicationAdd BLAST130

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AK022590 mRNA Translation: BAB14117.1
    AK222814 mRNA Translation: BAD96534.1
    AL356356 Genomic DNA No translation available.
    CH471121 Genomic DNA Translation: EAW53549.1
    BC000541 mRNA Translation: AAH00541.1
    BC007824 mRNA Translation: AAH07824.2
    BC009997 mRNA Translation: AAH09997.1
    CCDSiCCDS60252.1 [Q9BW92-2]
    CCDS952.1 [Q9BW92-1]
    RefSeqiNP_001258825.1, NM_001271896.1 [Q9BW92-2]
    NP_079426.2, NM_025150.4 [Q9BW92-1]
    UniGeneiHs.288974

    Genome annotation databases

    EnsembliENST00000369054; ENSP00000358050; ENSG00000143374 [Q9BW92-2]
    ENST00000369064; ENSP00000358060; ENSG00000143374 [Q9BW92-1]
    GeneIDi80222
    KEGGihsa:80222
    UCSCiuc001euq.5 human [Q9BW92-1]

    Keywords - Coding sequence diversityi

    Alternative splicing

    Similar proteinsi

    Entry informationi

    Entry nameiSYTM_HUMAN
    AccessioniPrimary (citable) accession number: Q9BW92
    Secondary accession number(s): Q53GW7, Q96I50, Q9H9V2
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 31, 2006
    Last sequence update: June 1, 2001
    Last modified: June 20, 2018
    This is version 153 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

    Complete proteome, Reference proteome

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