Proteins/UNIPROT:P49590

HARS2

ProteinUNIPROT:P49590Histidine--tRNA ligase, mitochondrial

Mitochondrial aminoacyl-tRNA synthetase that catalyzes the ATP-dependent ligation of histidine to the 3'-end of its cognate tRNA, via the formation of an aminoacyl-adenylate intermediate (His-AMP)..

Function from UniProtKB (P49590), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

1 row

Perrault syndrome 2 has a germline basis in HARS2DiseaseGenCC HGNC:4817|MONDO:0013972Inferred only

Statements

21 statements

21 statements · 4 reference databases · Subject: HARS2 · Sources: Hetionet, Reactome, Rhea, WikiPathways

Order

BST2 interacts with HARS2ProteinHetionet hetionet:interacts:23438-684Inferred only
MRPL58 interacts with HARS2ProteinHetionet hetionet:interacts:23438-3396Inferred only
UBC interacts with HARS2ProteinHetionet hetionet:interacts:23438-7316Inferred only
HARS2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
HARS2 participates in Mitochondrial tRNA aminoacylationPathwayReactome R-HSA-379726Inferred only
HARS2 participates in Primary ovarian insufficiencyPathwayWikiPathways WP5461Inferred only
HARS2 participates in TranslationPathwayReactome R-HSA-72766Inferred only
HARS2 participates in tRNA AminoacylationPathwayReactome R-HSA-379724Inferred only
ACO2 decreases the expression of HARS2ProteinHetionet hetionet:regulates:50-23438Inferred only
BAD increases the expression of HARS2ProteinHetionet hetionet:regulates:572-23438Inferred only
CD58 increases the expression of HARS2ProteinHetionet hetionet:regulates:965-23438Inferred only
CHN1 increases the expression of HARS2ProteinHetionet hetionet:regulates:1123-23438Inferred only

Show the remaining 9

DLD decreases the expression of HARS2ProteinHetionet hetionet:regulates:1738-23438Inferred only
IL20 decreases the expression of HARS2ProteinHetionet hetionet:regulates:50604-23438Inferred only
PCNA decreases the expression of HARS2ProteinHetionet hetionet:regulates:5111-23438Inferred only
PRPF6 increases the expression of HARS2ProteinHetionet hetionet:regulates:24148-23438Inferred only
PUF60 decreases the expression of HARS2ProteinHetionet hetionet:regulates:22827-23438Inferred only
RHEB increases the expression of HARS2ProteinHetionet hetionet:regulates:6009-23438Inferred only
RRM1 decreases the expression of HARS2ProteinHetionet hetionet:regulates:6240-23438Inferred only
STAM2 decreases the expression of HARS2ProteinHetionet hetionet:regulates:10254-23438Inferred only
L-histidine is a substrate of HARS2CompoundRhea RHEA:17313Inferred only

Reactions

1 reaction

CHEBI:78442+L-histidine+ATP(4-)CHEBI:78527+AMP+diphosphate(3-)+hydron
Catalysed by HARS1 or HARS2
Reaction pageDirection not assertedBalanced
Rhea RHEA:17313CC BY 4.0Reference data

Other names

6 names

Resolves to: HARS2

Also called: HARSLHARSRHisRSHistidine--tRNA ligase-likeHistidyl-tRNA synthetaseHO3

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