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GeneE
3 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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TARS3
threonyl-tRNA synthetase 3
Chromosome 15 Β· 15q26.3
NCBI Gene: 123283Ensembl: ENSG00000185418.17HGNC: HGNC:24728UniProt: A2RTX5
28PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
protein bindingthreonyl-tRNA aminoacylationthreonine-tRNA ligase activitynucleuspernicious anemiaglycogen storage disease IIIglycogen storage disease VIglycogen storage disease IXd
✦AI Summary

TARS3 (also known as TARSL2) is a threonyl-tRNA synthetase-like protein that catalyzes the aminoacylation of threonine to tRNA(Thr) through a two-step reaction involving threonine activation by ATP and subsequent transfer to the tRNA acceptor end 1. The enzyme possesses an editing domain that removes incorrectly charged tRNA(Thr) at the post-transfer stage 1. Unlike the cytoplasmic threonyl-tRNA synthetase (TARS), TARS3 localizes to both the cytoplasm and nucleus, with nuclear localization mediated by a C-terminal nuclear localization sequence 1. TARS3 demonstrates aminoacylation and editing activities comparable to canonical threonyl-tRNA synthetases, though with distinct tRNA recognition and editing capacity profiles 1. TARS3 is ubiquitously expressed across mouse tissues 1 and functions as a component of the aminoacyl-tRNA synthetase core complex 2. Clinically, dysregulation of TARS3 is implicated in glioblastoma pathogenesis; miR-720-mediated downregulation of TARS3 promotes glioma migration and invasion, suggesting TARS3 may function as a tumor suppressor 3.

Sources cited
1
TARS3/TARSL2 has aminoacylation and editing activities, localizes to cytoplasm and nucleus, and is ubiquitously expressed in tissues
PMID: 29579307
2
TARS3/TARSL2 is downregulated via miR-720 in glioblastoma and suppression of TARS3 promotes glioma migration and invasion
PMID: 34024034
3
TARS3/TARSL2 is a component of the aminoacyl-tRNA synthetase core complex
PMID: 24312579
⚠Limited data available β€” This gene has 3 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜20
pernicious anemiaOpen Targets
0.11Weak
glycogen storage disease IIIOpen Targets
0.04Suggestive
glycogen storage disease VIOpen Targets
0.04Suggestive
glycogen storage disease IXdOpen Targets
0.03Suggestive
coronary artery disease, autosomal dominant 2Open Targets
0.03Suggestive
autosomal recessive limb-girdle muscular dystrophy type 2LOpen Targets
0.03Suggestive
Short stature, Dauber-Argente typeOpen Targets
0.03Suggestive
dopa-responsive dystonia due to sepiapterin reductase deficiencyOpen Targets
0.03Suggestive
gliomaOpen Targets
0.02Suggestive
Huntington diseaseOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
myositisOpen Targets
0.01Suggestive
ovarian cancerOpen Targets
0.01Suggestive
acute lymphoblastic leukemiaOpen Targets
0.01Suggestive
Antisynthetase syndromeOpen Targets
0.01Suggestive
Duchenne muscular dystrophyOpen Targets
0.01Suggestive
infectionOpen Targets
0.01Suggestive
gastric cancerOpen Targets
0.00Suggestive
Yersinia pestis infectious diseaseOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar β†—
Related Genes
DARS2Shared pathway100%RPS14Protein interaction99%TARS1Protein interaction95%HARS1Protein interaction91%IARS1Protein interaction91%KARS1Protein interaction91%
Tissue Expression6 tissues
Heart
100%
Brain
84%
Ovary
47%
Lung
24%
Liver
23%
Bone Marrow
21%
Gene Interaction Network
Click a node to explore
TARS3DARS2RPS14TARS1HARS1IARS1KARS1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt A2RTX5
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
1.09LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.89 [0.72–1.09]
RankingsWhere TARS3 stands among ~20K protein-coding genes
  • #12,459of 20,598
    Most Researched28
  • #11,132of 17,882
    Most Constrained (LOEUF)1.09
Genes detectedTARS3
Sources retrieved3 papers
Response timeβ€”
πŸ“„ Sources
3
1
A threonyl-tRNA synthetase-like protein has tRNA aminoacylation and editing activities.
PMID: 29579307
Nucleic Acids Res Β· 2018
1.00
2
miR-720 is a key regulator of glioma migration and invasion by controlling TARSL2 expression.
PMID: 34024034
Hum Cell Β· 2021
0.67
3
Reinvestigation of aminoacyl-tRNA synthetase core complex by affinity purification-mass spectrometry reveals TARSL2 as a potential member of the complex.
PMID: 24312579
PLoS One Β· 2013
0.33