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Proteins/UNIPROT:Q5ST30

VARS2

Protein·UNIPROT:Q5ST30Valine--tRNA ligase, mitochondrial1 compound

Open in the graph

Catalyzes the attachment of valine to tRNA(Val) in a two-step reaction: valine is first activated by ATP to form Val-AMP and then transferred to the acceptor end of tRNA(Val)..

Function from UniProtKB (Q5ST30), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 1 · substrate of

Inferred

Assembled routes, not reported.

What routes through it

1 compound

1 compound.

0 reported routes1 assembled route

L-valineCompoundCHEBI:16414Inferred only4 paths4 endpoints

What the reference databases state

GenCC submitters' classifications

2 rows

combined oxidative phosphorylation defect type 20 has a germline basis in VARS2DiseaseGenCC HGNC:21642|MONDO:0014397Inferred only
mitochondrial disease has a germline basis in VARS2DiseaseGenCC HGNC:21642|MONDO:0044970Inferred only

Statements

8 statements

8 statements · 3 reference databases · Subject: VARS2 · Sources: Hetionet, Reactome, Rhea

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MRPL58 interacts with VARS2ProteinHetionet hetionet:interacts:3396-57176Inferred only
PIK3CA interacts with VARS2ProteinHetionet hetionet:interacts:5290-57176Inferred only
UBC interacts with VARS2ProteinHetionet hetionet:interacts:57176-7316Inferred only
VARS2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
VARS2 participates in Mitochondrial tRNA aminoacylationPathwayReactome R-HSA-379726Inferred only
VARS2 participates in TranslationPathwayReactome R-HSA-72766Inferred only
VARS2 participates in tRNA AminoacylationPathwayReactome R-HSA-379724Inferred only
L-valine is a substrate of VARS2CompoundRhea RHEA:10704Inferred only

Reactions

1 reaction

CHEBI:78442+L-valine+ATP(4-)CHEBI:78537+AMP+diphosphate(3-)
Catalysed by VARS1 or VARS2
Reaction pageLeft to rightBalanced
Rhea RHEA:10704CC BY 4.0Reference data

Other names

6 names

Resolves to: VARS2

Also called: KIAA1885ValRSValyl-tRNA synthetaseValyl-tRNA synthetase-likeVARS2LVARSL

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