Proteins/UNIPROT:Q7L3T8

PARS2

ProteinUNIPROT:Q7L3T8Probable proline--tRNA ligase, mitochondrial

Mitochondrial aminoacyl-tRNA synthetase that catalyzes the specific attachment of the proline amino acid (aa) to the homologous transfer RNA (tRNA), further participating in protein synthesis. The reaction occurs in a two steps: proline is first activated by ATP to form Pro-AMP and then transferred to the acceptor end of tRNA(Pro)..

Function from UniProtKB (Q7L3T8), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

3 rows

developmental and epileptic encephalopathy, 75 has a germline basis in PARS2DiseaseGenCC HGNC:30563|MONDO:0032752Inferred only
mitochondrial disease has a germline basis in PARS2DiseaseGenCC HGNC:30563|MONDO:0044970Inferred only
undetermined early-onset epileptic encephalopathy has a germline basis in PARS2DiseaseGenCC HGNC:30563|MONDO:0018614Inferred only

Statements

22 statements

22 statements · 3 reference databases · Subject: PARS2 · Sources: Hetionet, Reactome, Rhea

Order

ADAMTSL4 covaries with PARS2ProteinHetionet hetionet:covaries:25973-54507Inferred only
ASCL2 covaries with PARS2ProteinHetionet hetionet:covaries:25973-430Inferred only
ASPDH covaries with PARS2ProteinHetionet hetionet:covaries:25973-554235Inferred only
BOLA1 covaries with PARS2ProteinHetionet hetionet:covaries:25973-51027Inferred only
CASP12 covaries with PARS2ProteinHetionet hetionet:covaries:100506742-25973Inferred only
CYSRT1 covaries with PARS2ProteinHetionet hetionet:covaries:25973-375791Inferred only
EPOP covaries with PARS2ProteinHetionet hetionet:covaries:100170841-25973Inferred only
FOXJ2 covaries with PARS2ProteinHetionet hetionet:covaries:25973-55810Inferred only
FRAT2 covaries with PARS2ProteinHetionet hetionet:covaries:23401-25973Inferred only
MRGPRF covaries with PARS2ProteinHetionet hetionet:covaries:116535-25973Inferred only
PARS2 covaries with PERM1ProteinHetionet hetionet:covaries:25973-84808Inferred only
PARS2 covaries with VWA1ProteinHetionet hetionet:covaries:25973-64856Inferred only

Show the remaining 10

WHAMM covaries with PARS2ProteinHetionet hetionet:covaries:123720-25973Inferred only
WNT7B covaries with PARS2ProteinHetionet hetionet:covaries:25973-7477Inferred only
ZNF296 covaries with PARS2ProteinHetionet hetionet:covaries:162979-25973Inferred only
MRPL58 interacts with PARS2ProteinHetionet hetionet:interacts:25973-3396Inferred only
UBC interacts with PARS2ProteinHetionet hetionet:interacts:25973-7316Inferred only
PARS2 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
PARS2 participates in Mitochondrial tRNA aminoacylationPathwayReactome R-HSA-379726Inferred only
PARS2 participates in TranslationPathwayReactome R-HSA-72766Inferred only
PARS2 participates in tRNA AminoacylationPathwayReactome R-HSA-379724Inferred only
L-proline is a substrate of PARS2CompoundRhea RHEA:14305Inferred only

Reactions

1 reaction

CHEBI:78442+L-proline+ATP(4-)CHEBI:78532+AMP+diphosphate(3-)
Catalysed by EPRS1 or PARS2
Reaction pageLeft to rightBalanced
Rhea RHEA:14305CC BY 4.0Reference data

Other names

3 names

Resolves to: PARS2

Also called: Prolyl-tRNA synthetaseProlyl-tRNA synthetase 2, mitochondrialProRS

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