Proteins/UNIPROT:Q969Y2

GTPBP3

ProteinUNIPROT:Q969Y2

GTPase component of the GTPBP3-MTO1 complex that catalyzes the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position (U34) of mitochondrial tRNAs (mt-tRNAs), which plays a role in mt-tRNA decoding and mitochondrial translation. Taum(5)U formation on mammalian mt-tRNA requires the presence of both GTPBP3-mediated GTPase activity and MTO1 catalytic activity..

Function from UniProtKB (Q969Y2), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

3 rows

combined oxidative phosphorylation defect type 23 has a germline basis in GTPBP3DiseaseGenCC HGNC:14880|MONDO:0014525Inferred only
Leigh syndrome has a germline basis in GTPBP3DiseaseGenCC HGNC:14880|MONDO:0009723Inferred only
mitochondrial disease has a germline basis in GTPBP3DiseaseGenCC HGNC:14880|MONDO:0044970Inferred only

Statements

23 statements

23 statements · 4 reference databases · Subject: GTPBP3 · Sources: Hetionet, Reactome, UniProt, WikiPathways

Order

GTPBP3 covaries with COPRSProteinHetionet hetionet:covaries:55352-84705Inferred only
GTPBP3 covaries with CTSFProteinHetionet hetionet:covaries:84705-8722Inferred only
GTPBP3 covaries with PCDHB6ProteinHetionet hetionet:covaries:56130-84705Inferred only
GTPBP3 covaries with TCEAL4ProteinHetionet hetionet:covaries:79921-84705Inferred only
GTPBP3 covaries with UCN2ProteinHetionet hetionet:covaries:84705-90226Inferred only
CIC interacts with GTPBP3ProteinHetionet hetionet:interacts:23152-84705Inferred only
DCAF8 interacts with GTPBP3ProteinHetionet hetionet:interacts:50717-84705Inferred only
PARP11 interacts with GTPBP3ProteinHetionet hetionet:interacts:57097-84705Inferred only
PRPF3 interacts with GTPBP3ProteinHetionet hetionet:interacts:84705-9129Inferred only
PTAFR interacts with GTPBP3ProteinHetionet hetionet:interacts:5724-84705Inferred only
RIBC2 interacts with GTPBP3ProteinHetionet hetionet:interacts:26150-84705Inferred only
SINHCAF interacts with GTPBP3ProteinHetionet hetionet:interacts:58516-84705Inferred only

Show the remaining 11

UBC interacts with GTPBP3ProteinHetionet hetionet:interacts:7316-84705Inferred only
GTPBP3 participates in Metabolism of RNAPathwayReactome R-HSA-8953854Inferred only
GTPBP3 participates in miR targeted genes in lymphocytesPathwayWikiPathways WP2004Inferred only
GTPBP3 participates in tRNA modification in the mitochondrionPathwayReactome R-HSA-6787450Inferred only
GTPBP3 participates in tRNA processingPathwayReactome R-HSA-72306Inferred only
CDCA4 decreases the expression of GTPBP3ProteinHetionet hetionet:regulates:55038-84705Inferred only
FBXO7 decreases the expression of GTPBP3ProteinHetionet hetionet:regulates:25793-84705Inferred only
IKBKB increases the expression of GTPBP3ProteinHetionet hetionet:regulates:3551-84705Inferred only
KDM5A decreases the expression of GTPBP3ProteinHetionet hetionet:regulates:5927-84705Inferred only
STAMBP decreases the expression of GTPBP3ProteinHetionet hetionet:regulates:10617-84705Inferred only
GTPBP3 requires the cofactor K(+)CompoundUniProt Q969Y2Inferred only

Reactions

1 reaction

GTP+waterGDP+hydrogenphosphate+hydron
Catalysed by IRGM or RAB19 or TUBAL3 or GTPBP1 or RAB27B or RHOD or DNM1L or MRAS or RAB29 or OPA1 or EIF5B or ENTPD3 and 183 more alternatives
Reaction page

Other names

4 names

Resolves to: GTPBP3

Also called: GTP-binding protein 3Mitochondrial GTP-binding protein 1MTGP1tRNA modification GTPase GTPBP3, mitochondrial

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