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Protein

Threonine--tRNA ligase, mitochondrial

Gene

TARS2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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
    • RNA binding Source: GO_Central
    • 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

    HGNCiHGNC:30740. TARS2.

    Subcellular locationi

    GO - Cellular componenti

    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:rs587777593Ensembl.1

    Keywords - Diseasei

    Disease mutation

    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.

    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

    GO - Molecular functioni

    • protein homodimerization activity Source: UniProtKB

    Protein-protein interaction databases

    BioGridi123188. 12 interactors.
    IntActiQ9BW92. 9 interactors.
    MINTiMINT-8052579.
    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.
    OMAiTVYGCGM.
    OrthoDBiEOG093709FR.
    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.
    IPR012675. Beta-grasp_dom.
    IPR004095. TGS.
    IPR012676. TGS-like.
    IPR002320. Thr-tRNA-ligase_IIa.
    IPR018163. Thr/Ala-tRNA-synth_IIc_edit.
    IPR033728. ThrRS_core.
    IPR012947. tRNA_SAD.
    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.
    SUPFAMiSSF52954. SSF52954. 1 hit.
    SSF55186. 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:rs587777593Ensembl.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. Translation: CAI15488.1.
    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

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    AK022590 mRNA. Translation: BAB14117.1.
    AK222814 mRNA. Translation: BAD96534.1.
    AL356356 Genomic DNA. Translation: CAI15488.1.
    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.

    3D structure databases

    ProteinModelPortaliQ9BW92.
    SMRiQ9BW92.
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    BioGridi123188. 12 interactors.
    IntActiQ9BW92. 9 interactors.
    MINTiMINT-8052579.
    STRINGi9606.ENSP00000358060.

    Chemistry databases

    ChEMBLiCHEMBL3351186.
    DrugBankiDB00156. L-Threonine.

    PTM databases

    iPTMnetiQ9BW92.
    PhosphoSitePlusiQ9BW92.

    Polymorphism and mutation databases

    DMDMi74752395.

    Proteomic databases

    EPDiQ9BW92.
    MaxQBiQ9BW92.
    PaxDbiQ9BW92.
    PeptideAtlasiQ9BW92.
    PRIDEiQ9BW92.

    Protocols and materials databases

    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

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

    Organism-specific databases

    CTDi80222.
    DisGeNETi80222.
    GeneCardsiMIR6878.
    TARS2.
    HGNCiHGNC:30740. TARS2.
    HPAiHPA028626.
    MalaCardsiTARS2.
    MIMi612805. gene.
    615918. phenotype.
    neXtProtiNX_Q9BW92.
    OpenTargetsiENSG00000143374.
    PharmGKBiPA162405200.
    GenAtlasiSearch...

    Phylogenomic databases

    eggNOGiKOG1637. Eukaryota.
    COG0441. LUCA.
    GeneTreeiENSGT00390000002149.
    HOGENOMiHOG000003878.
    HOVERGENiHBG059513.
    InParanoidiQ9BW92.
    KOiK01868.
    OMAiTVYGCGM.
    OrthoDBiEOG093709FR.
    PhylomeDBiQ9BW92.
    TreeFamiTF300858.

    Enzyme and pathway databases

    ReactomeiR-HSA-379726. Mitochondrial tRNA aminoacylation.

    Miscellaneous databases

    ChiTaRSiTARS2. human.
    GenomeRNAii80222.
    PROiQ9BW92.
    SOURCEiSearch...

    Gene expression databases

    BgeeiENSG00000143374.
    CleanExiHS_TARS2.
    ExpressionAtlasiQ9BW92. baseline and differential.
    GenevisibleiQ9BW92. HS.

    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.
    IPR012675. Beta-grasp_dom.
    IPR004095. TGS.
    IPR012676. TGS-like.
    IPR002320. Thr-tRNA-ligase_IIa.
    IPR018163. Thr/Ala-tRNA-synth_IIc_edit.
    IPR033728. ThrRS_core.
    IPR012947. tRNA_SAD.
    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.
    SUPFAMiSSF52954. SSF52954. 1 hit.
    SSF55186. SSF55186. 1 hit.
    SSF81271. SSF81271. 1 hit.
    TIGRFAMsiTIGR00418. thrS. 1 hit.
    PROSITEiView protein in PROSITE
    PS50862. AA_TRNA_LIGASE_II. 1 hit.
    ProtoNetiSearch...

    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: February 15, 2017
    This is version 141 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

    Documents

    1. Aminoacyl-tRNA synthetases
      List of aminoacyl-tRNA synthetase entries
    2. Human chromosome 1
      Human chromosome 1: 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. SIMILARITY comments
      Index of protein domains and families

    Similar proteinsi

    Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
    100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
    90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
    50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.