3 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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28PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITYβ Experimental GO Evidenceβ Swiss-Prot Reviewed
protein bindingthreonyl-tRNA aminoacylationthreonine-tRNA ligase activitynucleuspernicious anemiaglycogen storage disease IIIglycogen storage disease VIglycogen storage disease IXd
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.
1
TARS3/TARSL2 has aminoacylation and editing activities, localizes to cytoplasm and nucleus, and is ubiquitously expressed in tissues
PMID: 295793072
TARS3/TARSL2 is downregulated via miR-720 in glioblastoma and suppression of TARS3 promotes glioma migration and invasion
PMID: 340240343
TARS3/TARSL2 is a component of the aminoacyl-tRNA synthetase core complex
PMID: 24312579β Limited data available β This gene has 3 indexed publications. Summary and analysis may be incomplete.
pernicious anemiaOpen Targets
glycogen storage disease IIIOpen Targets
glycogen storage disease VIOpen Targets
glycogen storage disease IXdOpen Targets
coronary artery disease, autosomal dominant 2Open Targets
autosomal recessive limb-girdle muscular dystrophy type 2LOpen Targets
Short stature, Dauber-Argente typeOpen Targets
dopa-responsive dystonia due to sepiapterin reductase deficiencyOpen Targets
Huntington diseaseOpen Targets
ovarian cancerOpen Targets
acute lymphoblastic leukemiaOpen Targets
Antisynthetase syndromeOpen Targets
Duchenne muscular dystrophyOpen Targets
gastric cancerOpen Targets
Yersinia pestis infectious diseaseOpen Targets
No pathogenic variants reported on ClinVar for this gene.