gtpbp3

UniProt ID: Q501Z5
Organism: Danio rerio
Review Status: INITIALIZED
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Gene Description

gtpbp3 encodes a mitochondrial GTPase subunit of the GTPBP3-MTO1 complex required for taurine-containing wobble uridine (tau-5-methyluridine) modification of mitochondrial tRNAs. Its GTPase activity drives this tRNA modification; loss impairs mitochondrial translation, with reduced mitochondrial tRNA aminoacylation arising as a downstream consequence rather than from a direct aminoacyl-tRNA ligase activity of the protein.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0005737 cytoplasm
IBA
GO_REF:0000033
REMOVE
Summary: cytoplasm (GO:0005737) is too coarse for gtpbp3, whose specific mitochondrion location is annotated separately.
Reason: gtpbp3 is a mitochondrial tRNA-modifying enzyme. Mitochondrion (GO:0005739) is part_of cytoplasm and is annotated directly for this gene, so the transferred cytoplasm term is a true-but-uninformative parent and is removed as redundant rather than retained.
Propagation Review
Root cause: TERM SCOPING PROBLEM
Failure modes: GRANULARITY MISMATCH
Sources checked:
PANTHER:PTN000182687 Β· GTPBP3/MnmE tRNA-modifying GTPase family node SUPPORTS TRANSFER
gtpbp3 is a mitochondrial tRNA-modifying enzyme. GO defines mitochondrion (GO:0005739) as part_of cytoplasm, so the transferred cytoplasm term is technically true but too coarse; it is removed as redundant with the specific mitochondrion annotation reviewed separately.
SGD:S000004625 Β· yeast MSS1 mitochondrial GTPase donor SUPPORTS TRANSFER
Donor supports the localization; the specific mitochondrion term supersedes the vague cytoplasm parent for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0002098 tRNA wobble uridine modification
IBA
GO_REF:0000033
ACCEPT
Summary: tRNA wobble uridine modification (GO:0002098) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
Across mechanistic summaries, GTPBP3 functions together with MTO1 in the **biosynthesis of Ο„m5U/Ο„m5(s2)U at mitochondrial tRNA wobble uridine (U34)**
GO:0005739 mitochondrion
IBA
GO_REF:0000033
ACCEPT
Summary: mitochondrion (GO:0005739) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0030488 tRNA methylation
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: tRNA methylation (GO:0030488) is retained as supported context for gtpbp3 but is not the primary/core function.
Reason: This annotation is broad, inferred, or reflects downstream developmental/physiological context rather than the central molecular role.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0003924 GTPase activity
IEA
GO_REF:0000002
ACCEPT
Summary: GTPase activity (GO:0003924) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
GTPBP3 is described as a **nuclear-encoded mitochondrial tRNA-modifying enzyme** with an **atypical TrmE-type GTPase** domain where **GTP hydrolysis is functionally important**
GO:0005525 GTP binding
IEA
GO_REF:0000002
ACCEPT
Summary: GTP binding (GO:0005525) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0005739 mitochondrion
IEA
GO_REF:0000120
ACCEPT
Summary: mitochondrion (GO:0005739) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0006400 tRNA modification
IEA
GO_REF:0000002
KEEP AS NON CORE
Summary: tRNA modification (GO:0006400) is retained as supported context for gtpbp3 but is not the primary/core function.
Reason: This annotation is broad, inferred, or reflects downstream developmental/physiological context rather than the central molecular role.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070900 mitochondrial tRNA modification
IEA
GO_REF:0000117
ACCEPT
Summary: mitochondrial tRNA modification (GO:0070900) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0003924 GTPase activity
ISS
GO_REF:0000024
ACCEPT
Summary: GTPase activity (GO:0003924) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0005739 mitochondrion
ISS
GO_REF:0000024
ACCEPT
Summary: mitochondrion (GO:0005739) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070899 mitochondrial tRNA wobble uridine modification
ISS
GO_REF:0000024
ACCEPT
Summary: mitochondrial tRNA wobble uridine modification (GO:0070899) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
file:DANRE/gtpbp3/gtpbp3-uniprot.txt
the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
Across mechanistic summaries, GTPBP3 functions together with MTO1 in the **biosynthesis of Ο„m5U/Ο„m5(s2)U at mitochondrial tRNA wobble uridine (U34)**
GO:0036416 tRNA stabilization
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
KEEP AS NON CORE
Summary: tRNA stabilization (GO:0036416) is retained as supported context for gtpbp3 but is not the primary/core function.
Reason: This annotation is broad, inferred, or reflects downstream developmental/physiological context rather than the central molecular role.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070143 mitochondrial alanyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial alanyl-tRNA aminoacylation (GO:0070143) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function (wobble U34 modification) or downstream phenotype; this term should not be treated as a direct/core annotation. The falcon synthesis reinforces this: gtpbp3-knockout zebrafish showed increased (not abolished) tRNA aminoacylation efficiencies, which is inconsistent with gtpbp3 acting as a direct aminoacyl-tRNA ligase and instead reflects an indirect consequence of altered tRNA modification.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
gtpbp3KO zebrafish showed increased efficiencies of tRNA aminoacylation
GO:0070153 mitochondrial leucyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial leucyl-tRNA aminoacylation (GO:0070153) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function or downstream phenotype; this term should not be treated as a direct/core annotation.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070154 mitochondrial lysyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial lysyl-tRNA aminoacylation (GO:0070154) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function or downstream phenotype; this term should not be treated as a direct/core annotation.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070155 mitochondrial methionyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial methionyl-tRNA aminoacylation (GO:0070155) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function or downstream phenotype; this term should not be treated as a direct/core annotation.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070183 mitochondrial tryptophanyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial tryptophanyl-tRNA aminoacylation (GO:0070183) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function or downstream phenotype; this term should not be treated as a direct/core annotation.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070184 mitochondrial tyrosyl-tRNA aminoacylation
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
MARK AS OVER ANNOTATED
Summary: mitochondrial tyrosyl-tRNA aminoacylation (GO:0070184) likely overstates or misdirects the direct function of gtpbp3.
Reason: The evidence is better explained by the gene core function or downstream phenotype; this term should not be treated as a direct/core annotation.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
GO:0070900 mitochondrial tRNA modification
IMP
PMID:30916346
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic ca...
ACCEPT
Summary: mitochondrial tRNA modification (GO:0070900) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
PMID:30916346
GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
the **phenotype (cardiomyopathy) + aberrant mitochondrial tRNA metabolism** strongly supports conserving the canonical mitochondrial tRNA wobble-modification function for zebrafish Gtpbp3
GO:0005739 mitochondrion
IDA
PMID:27184967
The defective expression of gtpbp3 related to tRNA modificat...
ACCEPT
Summary: mitochondrion (GO:0005739) is supported for gtpbp3.
Reason: This annotation matches the synthesized core function or a directly supported core location/process for this gene.
Supporting Evidence:
PMID:27184967
Zebrafish gtpbp3 has three isoforms localized at mitochondria
file:DANRE/gtpbp3/gtpbp3-deep-research-falcon.md
GTPBP3 is consistently described as **mitochondrial** (including presence of a mitochondrial targeting sequence and its placement in mitochondrial tRNA modification machinery)
GO:0048568 embryonic organ development
IMP
PMID:27184967
The defective expression of gtpbp3 related to tRNA modificat...
KEEP AS NON CORE
Summary: embryonic organ development (GO:0048568) is retained as supported context for gtpbp3 but is not the primary/core function.
Reason: This annotation is broad, inferred, or reflects downstream developmental/physiological context rather than the central molecular role.
Supporting Evidence:
PMID:27184967
Zebrafish gtpbp3 has three isoforms localized at mitochondria

Core Functions

Gtpbp3 uses GTPase activity in mitochondria to support wobble uridine modification of mitochondrial tRNAs.

Supporting Evidence:
  • file:DANRE/gtpbp3/gtpbp3-uniprot.txt
    the 5-taurinomethyluridine (taum(5)U) modification at the 34th wobble position
  • PMID:30916346
    GTPBP3 is a highly conserved tRNA modifying enzyme for the biosynthesis of Ο„m5U at the wobble position of mitochondrial tRNA

References

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Deep Research

Falcon

(gtpbp3-deep-research-falcon.md)

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πŸ“š Additional Documentation

Notes

(gtpbp3-notes.md)

gtpbp3 (Danio rerio) review notes

Batch 05 curation notes

  • Core interpretation: gtpbp3 encodes a mitochondrial GTPase subunit of the GTPBP3-MTO1 complex required for taurine-containing wobble uridine modification of mitochondrial tRNAs. The core function is GTPase-supported mitochondrial tRNA wobble uridine modification; reduced mitochondrial tRNA aminoacylation phenotypes are treated as downstream consequences rather than direct aminoacyl-tRNA ligase activity.
  • Key UniProt support: GTPase component of the GTPBP3-MTO1 complex.
  • Existing GOA annotations were reviewed against this core role; broad, inferred, or downstream phenotype annotations were kept as non-core unless they overstate the direct function.

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