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A reference for what the literature reports. Not medical advice.

© 2026 CatalystPrivacy TermsReference data under the licences listed on the attribution page.

Proteins/UNIPROT:Q5JPH6

EARS2

Protein·UNIPROT:Q5JPH6Nondiscriminating glutamyl-tRNA synthetase EARS2, mitochondrial

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Non-discriminating glutamyl-tRNA synthetase that catalyzes aminoacylation of both mitochondrial tRNA(Glu) and tRNA(Gln) and participates in RNA aminoacylation for mitochondrial protein translation. Attachs glutamate to tRNA(Glu) or tRNA(Gln) in a two-step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu) or tRNA(Gln). In vitro, cytoplasmic tRNA(Gln) is slightly glutamylated, but with low activity..

Function from UniProtKB (Q5JPH6), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

3 rows

Leigh syndrome has a germline basis in EARS2DiseaseGenCC HGNC:29419|MONDO:0009723Inferred only
leukoencephalopathy-thalamus and brainstem anomalies-high lactate syndrome has a germline basis in EARS2DiseaseGenCC HGNC:29419|MONDO:0013971Inferred only
mitochondrial disease has a germline basis in EARS2DiseaseGenCC HGNC:29419|MONDO:0044970Inferred only

Statements

1 statement

1 statement · 1 reference database · Subject: EARS2 · Sources: Hetionet

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UBC interacts with EARS2ProteinHetionet hetionet:interacts:124454-7316Inferred only

Reactions

3 reactions

CHEBI:78442+L-glutamate(1-)+ATP(4-)CHEBI:78520+AMP+diphosphate(3-)
Catalysed by EARS2
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:18397CC BY 4.0Reference data
CHEBI:78442+L-glutamate(1-)+ATP(4-)CHEBI:78520+AMP+diphosphate(3-)
Catalysed by EPRS1 or

Other names

6 names

Resolves to: EARS2

Also called: GluRSGlutamate--tRNA(Gln) ligase EARS2, mitochondrialGlutamyl-tRNA synthetaseKIAA1970Mitochondrial glutamyl-tRNA synthetasemtGluRS

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Reaction pageLeft to rightBalance undeterminable

Rhea RHEA:23540CC BY 4.0Reference data

CHEBI:78442+L-glutamate(1-)+ATP(4-)CHEBI:78520+AMP+diphosphate(3-)

Catalysed by EARS2

Reaction pageLeft to rightBalance undeterminable

Rhea RHEA:64612CC BY 4.0Reference data