Proteins/UNIPROT:Q9BW92

TARS2

ProteinUNIPROT:Q9BW92Threonine--tRNA ligase, mitochondrial

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..

Function from UniProtKB (Q9BW92), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

combined oxidative phosphorylation defect type 21 has a germline basis in TARS2DiseaseGenCC HGNC:30740|MONDO:0014398Inferred only
mitochondrial disease has a germline basis in TARS2DiseaseGenCC HGNC:30740|MONDO:0044970Inferred only

Statements

12 statements

12 statements · 3 reference databases · Subject: TARS2 · Sources: Hetionet, Reactome, Rhea

Order

CCDC86 covaries with TARS2ProteinHetionet hetionet:covaries:79080-80222Inferred only
OTUD1 covaries with TARS2ProteinHetionet hetionet:covaries:220213-80222Inferred only
VWA5B2 covaries with TARS2ProteinHetionet hetionet:covaries:80222-90113Inferred only
CUL3 interacts with TARS2ProteinHetionet hetionet:interacts:80222-8452Inferred only
MRPL58 interacts with TARS2ProteinHetionet hetionet:interacts:3396-80222Inferred only
UBC interacts with TARS2ProteinHetionet hetionet:interacts:7316-80222Inferred only
UBD interacts with TARS2ProteinHetionet hetionet:interacts:10537-80222Inferred only
TARS2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
TARS2 participates in Mitochondrial tRNA aminoacylationPathwayReactome R-HSA-379726Inferred only
TARS2 participates in TranslationPathwayReactome R-HSA-72766Inferred only
TARS2 participates in tRNA AminoacylationPathwayReactome R-HSA-379724Inferred only
L-threonine is a substrate of TARS2CompoundRhea RHEA:24624Inferred only

Reactions

1 reaction

CHEBI:78442+L-threonine+ATP(4-)CHEBI:78534+AMP+diphosphate(3-)+hydron
Catalysed by TARS3 or TARS1 or TARS2
Reaction pageDirection not assertedBalanced
Rhea RHEA:24624CC BY 4.0Reference data

Other names

4 names

Resolves to: TARS2

Also called: TARSL1Threonyl-tRNA synthetaseThreonyl-tRNA synthetase-like 1ThrRS

← Every protein Find it in the graph →