GPKOW

UniProt ID: Q92917
Organism: Homo sapiens
Review Status: COMPLETE
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Gene Description

GPKOW is a nuclear MOS2-family protein with G-patch and KOW domains. It binds RNA and DHX16/hPrp2 and participates in spliceosomal pre-mRNA processing. Pathogenic GPKOW variants cause X-linked developmental disorders involving growth, brain and eye abnormalities.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0000398 mRNA splicing, via spliceosome
IBA
GO_REF:0000033
ACCEPT
Reason: The Eukaryota IBD includes the target and legitimate self evidence; human splicing assays corroborate the inherited function.
Supporting Evidence:
file:human/GPKOW/GPKOW-evidence.json
GO:0000398 mRNA splicing, via spliceosome
IEA
GO_REF:0000002
ACCEPT
Reason: Human splicing experiments corroborate this electronic assignment.
Supporting Evidence:
GO:0000398 mRNA splicing, via spliceosome
IMP
PMID:25296192
GPKOW is essential for pre-mRNA splicing inΒ vitro and suppre...
ACCEPT
Reason: Depletion/add-back supports splicing, with partial rescue and DHX16 codepletion.
Supporting Evidence:
GO:0000398 mRNA splicing, via spliceosome
NAS
PMID:33509932
Structure of the activated human minor spliceosome.
ACCEPT
Reason: Minor-spliceosome membership supports participation; only GPKOW residues 18–30 are structurally resolved.
Supporting Evidence:
file:human/GPKOW/GPKOW-uniprot.txt
minor spliceosome, which splices U12-type
GO:0003676 nucleic acid binding
IEA
GO_REF:0000002
TOO GENERAL
Summary: Prefer the existing RNA-binding specificity.
Reason: The existing RNA-binding term is an is_a descendant of nucleic acid binding; it retains the informative specificity without a second broad binding assertion.
GO:0003723 RNA binding
IDA
PMID:21880142
G-patch domain and KOW motifs-containing protein, GPKOW; a n...
ACCEPT
Reason: Cellular RNA association is corroborated by purified-protein binding.
Supporting Evidence:
GO:0003723 RNA binding
IMP
PMID:25296192
GPKOW is essential for pre-mRNA splicing inΒ vitro and suppre...
ACCEPT
Reason: Purified-protein binding supports the retained IMP context.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:16491121
Smad6 is a protein kinase X phosphorylation substrate and is...
MODIFY
Reason: The abstract explicitly identifies T54 as a PRKX partner by two-hybrid and co-immunoprecipitation; refine to kinase binding.
Proposed replacements: protein kinase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:21044950
Genome-wide YFP fluorescence complementation screen identifi...
REMOVE
Reason: Retain the curated TERF2IP association; generic binding does not specify a GPKOW activity.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:21516116
Next-generation sequencing to generate interactome datasets.
REMOVE
Reason: Retain the curated SH3GL1 interaction from this human interactome screen; the generic term is uninformative.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:21880142
G-patch domain and KOW motifs-containing protein, GPKOW; a n...
MODIFY
Reason: Human CΞ²2 interacts with GPKOW in yeast and variably by co-immunoprecipitation. Refine to PKA catalytic-subunit binding without assigning kinase activity to GPKOW.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
REMOVE
Reason: Retain the curated spliceosomal interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
MODIFY
Reason: Targeted human hPRP2–GPKOW binding and competition assays support helicase binding; the proposed recruitment model does not establish obligatory helicase activation.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
REMOVE
Reason: Retain the curated spliceosomal interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
REMOVE
Reason: Retain the curated spliceosomal interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
MODIFY
Reason: Targeted human hPRP16–GPKOW competition assays support helicase binding, without establishing helicase activation or a fixed native recruitment sequence.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:22365833
Dynamic protein-protein interaction wiring of the human spli...
REMOVE
Reason: Retain the curated spliceosomal interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25296192
GPKOW is essential for pre-mRNA splicing inΒ vitro and suppre...
MODIFY
Reason: Mutant-sensitive, RNase-resistant DHX16 binding supports this refinement; DHX16 was supplied in cell lysate.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified TRIM23 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q08379 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q12933 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q15560 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q2TAC2 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q6FGM0 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified BUD13 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:25416956
A proteome-scale map of the human interactome network.
REMOVE
Reason: Retain the verified Q9HAT0 two-hybrid interaction; remove the uninformative term.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:28514442
Architecture of the human interactome defines protein commun...
MODIFY
Reason: Retain DHX16 co-complex association; targeted experiments independently support helicase binding, without establishing activation.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:28514442
Architecture of the human interactome defines protein commun...
REMOVE
Reason: Retain the BUD13 AP-MS association; the generic term adds no informative molecular function.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:30021884
Histone Interaction Landscapes Visualized by Crosslinking Ma...
REMOVE
Reason: Retain nuclear HNRNPC crosslinking evidence and its extension; the generic binding term is uninformative.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:31515488
Extensive disruption of protein interactions by genetic vari...
REMOVE
Reason: Retain the TRIM23 interaction; the variant-screen record does not assign a mutant identity to this unsuffixed annotation.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:31515488
Extensive disruption of protein interactions by genetic vari...
REMOVE
Reason: Retain the Q08379 interaction; remove the uninformative term without projecting variant effects onto canonical GPKOW.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:31515488
Extensive disruption of protein interactions by genetic vari...
REMOVE
Reason: Retain the Q12933 interaction; remove the uninformative term without projecting variant effects onto canonical GPKOW.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:31515488
Extensive disruption of protein interactions by genetic vari...
REMOVE
Reason: Retain the Q15560 interaction; remove the uninformative term without projecting variant effects onto canonical GPKOW.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:31515488
Extensive disruption of protein interactions by genetic vari...
REMOVE
Reason: Retain the BUD13 interaction; remove the uninformative term without projecting variant effects onto canonical GPKOW.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:32296183
A reference map of the human binary protein interactome.
REMOVE
Reason: Uninformative term; interaction retained.
Supporting Evidence:
file:human/GPKOW/GPKOW-source-guide.md
GO:0005515 protein binding
IPI
PMID:33961781
Dual proteome-scale networks reveal cell-specific remodeling...
MODIFY
Reason: Retain the cell-specific DHX16 associations; targeted experiments independently justify helicase binding.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:33961781
Dual proteome-scale networks reveal cell-specific remodeling...
REMOVE
Reason: Retain the BUD13 co-complex association; remove the uninformative term without claiming binary binding.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:39251607
Systematic identification of post-transcriptional regulatory...
MODIFY
Reason: Proximity labeling is not binary binding; targeted DHX16 experiments independently justify the helicase-binding refinement.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:39251607
Systematic identification of post-transcriptional regulatory...
REMOVE
Reason: Retain the HNRNPC proximity and nuclear extension; remove the uninformative term without inferring direct contact.
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:40205054
Multimodal cell maps as a foundation for structural and func...
MODIFY
Reason: The original U2OS network contains DHX16; targeted experiments independently ground helicase binding.
Proposed replacements: DEAD/H-box RNA helicase binding
Supporting Evidence:
GO:0005515 protein binding
IPI
PMID:40205054
Multimodal cell maps as a foundation for structural and func...
REMOVE
Reason: Retain the original U2OS GPKOW–BUD13 edge; remove the uninformative term without inferring direct contact.
Supporting Evidence:
GO:0005634 nucleus
IDA
PMID:21880142
G-patch domain and KOW motifs-containing protein, GPKOW; a n...
TOO GENERAL
Reason: The accepted located_in nucleoplasm annotation entails located_in nucleus through the part_of relation. These rows concern the same canonical product without distinct extensions; retain the more specific localization.
GO:0005634 nucleus
IDA
PMID:25296192
GPKOW is essential for pre-mRNA splicing inΒ vitro and suppre...
TOO GENERAL
Reason: The accepted located_in nucleoplasm annotation entails located_in nucleus through the part_of relation. These rows concern the same canonical product without distinct extensions; retain the more specific localization.
GO:0005634 nucleus
IEA
GO_REF:0000044
TOO GENERAL
Reason: The accepted located_in nucleoplasm annotation entails located_in nucleus through the part_of relation. These rows concern the same canonical product without distinct extensions; retain the more specific localization.
GO:0005654 nucleoplasm
IDA
GO_REF:0000052
ACCEPT
Summary: Retain the nucleoplasm localization.
Reason: The current HPA page reports enhanced-reliability nucleoplasmic localization with HPA000287 and HPA001894 in A-431, U-251MG and U2OS cells. No independent image reanalysis is claimed.
Supporting Evidence:
GO_REF:0000052
GO:0005654 nucleoplasm
TAS
Reactome:R-HSA-9770131
ACCEPT
Reason: The event contains human Q92917 in the nucleoplasmic Bact input and as a released nucleoplasmic output. Its exact GPKOW entity supports this location; release does not establish membership in the B-star output complex.
Supporting Evidence:
Reactome:R-HSA-9770131
file:human/GPKOW/GPKOW-evidence.json
GO:0005654 nucleoplasm
TAS
Reactome:R-HSA-9770145
ACCEPT
Reason: Human Q92917 is an explicit nucleoplasmic input and a component of the nucleoplasmic Bact output. The target entity itself supports this location, rather than only the compartment of a parent event.
Supporting Evidence:
Reactome:R-HSA-9770145
file:human/GPKOW/GPKOW-evidence.json
GO:0005654 nucleoplasm
TAS
Reactome:R-HSA-9921507
ACCEPT
Reason: The nucleoplasmic input complex and NS5-bound output both resolve to the human Q92917 entity in the nucleoplasm. This supports the location annotation without asserting direct GPKOW-NS5 binding.
Supporting Evidence:
Reactome:R-HSA-9921507
file:human/GPKOW/GPKOW-evidence.json
GO:0005681 spliceosomal complex
IBA
GO_REF:0000033
ACCEPT
Reason: Retain broad spliceosomal membership: human evidence covers major and minor assemblies, and U12 membership does not replace the major-spliceosome context.
Supporting Evidence:
file:human/GPKOW/GPKOW-evidence.json
GO:0005681 spliceosomal complex
IDA
PMID:25296192
GPKOW is essential for pre-mRNA splicing inΒ vitro and suppre...
ACCEPT
Reason: GPKOW associates with pre-mRNA/intermediates during major B/C-complex assembly.
Supporting Evidence:
GO:0071018 U12-type catalytic step 2 spliceosome
IPI
PMID:33509932
Structure of the activated human minor spliceosome.
MODIFY
Reason: GPKOW residues 18–30 occur in pre-branching Bact. Step 2 requires prior cleavage; step 1 defines catalytically active B*. Preserve U12-complex membership pending stage clarification.
Proposed replacements: U12-type spliceosomal complex
Supporting Evidence:
file:human/GPKOW/GPKOW-evidence.json
file:human/GPKOW/GPKOW-uniprot.txt
minor spliceosome, which splices U12-type

Core Functions

RNA binding supports spliceosomal pre-mRNA processing.

Molecular Function:
RNA binding
Directly Involved In:
Cellular Locations:
Supporting Evidence:

G-patch-dependent DHX16 binding accompanies splicing. A binding-defective mutant retained rescue in human extracts, so obligatory recruitment or helicase activation is not established.

Cellular Locations:
Supporting Evidence:

References

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Suggested Questions for Experts

Q: Which GO term covers the pre-Prp2 minor Bact state in CPX-26509?

Suggested experts: GO and ComplexPortal curators

πŸ“š Additional Documentation

Notes

(GPKOW-notes.md)

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Source Guide

(GPKOW-source-guide.md)

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πŸ“„ View Raw YAML

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