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Proteins/UNIPROT:Q5T160

RARS2

Protein·UNIPROT:Q5T160Probable arginine--tRNA ligase, mitochondrial1 compound

Open in the graph

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

Function from UniProtKB (Q5T160), 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-arginineCompoundCHEBI:16467Inferred only4 paths4 endpoints

What the reference databases state

GenCC submitters' classifications

2 rows

mitochondrial disease has a germline basis in RARS2DiseaseGenCC HGNC:21406|MONDO:0044970Inferred only
pontocerebellar hypoplasia type 6 has a germline basis in RARS2DiseaseGenCC HGNC:21406|MONDO:0012683Inferred only

Statements

9 statements

9 statements · 4 reference databases · Subject: RARS2 · Sources: Bgee, Hetionet, Reactome, Rhea

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RARS2 is expressed in ileal mucosaAnatomyBgee ENSG00000146282Inferred only
RARS2 is expressed in tibiaAnatomyBgee ENSG00000146282Inferred only
RARS2 is expressed in tibialis anteriorAnatomyBgee ENSG00000146282Inferred only
UBC interacts with RARS2ProteinHetionet hetionet:interacts:57038-7316Inferred only
RARS2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
RARS2 participates in Mitochondrial tRNA aminoacylationPathwayReactome R-HSA-379726Inferred only
RARS2 participates in TranslationPathwayReactome R-HSA-72766Inferred only
RARS2 participates in tRNA AminoacylationPathwayReactome R-HSA-379724Inferred only
L-arginine is a substrate of RARS2CompoundRhea RHEA:20301Inferred only

Reactions

1 reaction

CHEBI:78442+L-arginine+ATP(4-)CHEBI:78513+AMP+diphosphate(3-)
Catalysed by RARS1 or RARS2
Reaction pageDirection not assertedBalanced
Rhea RHEA:20301CC BY 4.0Reference data

Other names

3 names

Resolves to: RARS2

Also called: Arginyl-tRNA synthetaseArgRSRARSL

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