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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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GPN3
GPN-loop GTPase 3
Chromosome 12 · 12q24.11
NCBI Gene: 51184Ensembl: ENSG00000111231.10HGNC: HGNC:30186UniProt: Q9UHW5
53PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleusprotein bindingprotein import into nucleuscytoplasmpoisoningneurodegenerative diseaseAbnormality of the skeletal systemobesity
✦AI Summary

GPN3 is a small GTPase that plays essential roles in RNA polymerase II (RNAPII) biogenesis and nuclear import. GPN3 directly associates with RNAPII subunits RPB7/RPB4 and the C-terminal domain, facilitating nuclear translocation of the polymerase complex 1. The protein functions cooperatively with other GPN family members, particularly GPN1, with mutual dependency between their protein levels 2. GPN3 undergoes regulated polyubiquitination on lysine 216 in the nucleus, targeting it for proteasomal degradation in a GPN1-inhibitable manner 3. Beyond its canonical role in transcriptional machinery assembly, GPN3 regulates clathrin-mediated endocytosis by interacting with clathrin light chain A (CLTA) and associated proteins, affecting EGFR internalization and prolonging growth factor signaling 4. GPN3 is essential for cell proliferation across various breast cancer cell lines regardless of transformation level, with knockdown causing cytoplasmic retention of RNAPII subunits 5. The protein is upregulated in multiple cancer types including non-small cell lung cancer and small-cell lung cancer, where it contributes to MYC-dependent ribosome biogenesis programs 46. High GPN3 expression correlates with poor prognosis in several cancer types, suggesting its potential as both a biomarker and therapeutic target.

Sources cited
1
GPN3 directly associates with RNAPII subunits RPB7/RPB4 and the C-terminal domain, facilitating nuclear translocation of the polymerase complex .
PMID: 21768307
2
The protein functions cooperatively with other GPN family members, particularly GPN1, with mutual dependency between their protein levels .
PMID: 32985767
3
GPN3 undergoes regulated polyubiquitination on lysine 216 in the nucleus, targeting it for proteasomal degradation in a GPN1-inhibitable manner .
PMID: 29029378
4
Beyond its canonical role in transcriptional machinery assembly, GPN3 regulates clathrin-mediated endocytosis by interacting with clathrin light chain A (CLTA) and associated proteins, affecting EGFR internalization and prolonging growth factor signaling .
PMID: 39893205
5
GPN3 is essential for cell proliferation across various breast cancer cell lines regardless of transformation level, with knockdown causing cytoplasmic retention of RNAPII subunits .
PMID: 31431135
6
The protein is upregulated in multiple cancer types including non-small cell lung cancer and small-cell lung cancer, where it contributes to MYC-dependent ribosome biogenesis programs .
PMID: 33079728
Disease Associationsⓘ20
poisoningOpen Targets
0.27Weak
neurodegenerative diseaseOpen Targets
0.23Weak
Abnormality of the skeletal systemOpen Targets
0.10Weak
obesityOpen Targets
0.09Suggestive
schizophreniaOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
placenta praeviaOpen Targets
0.06Suggestive
HeadacheOpen Targets
0.03Suggestive
PainOpen Targets
0.03Suggestive
neoplasmOpen Targets
0.03Suggestive
atrial fibrillationOpen Targets
0.03Suggestive
hypertrophic cardiomyopathyOpen Targets
0.02Suggestive
lung adenocarcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Alzheimer diseaseOpen Targets
0.01Suggestive
meningococcal infectionOpen Targets
0.00Suggestive
IGA glomerulonephritisOpen Targets
0.00Suggestive
small cell lung carcinomaOpen Targets
0.00Suggestive
PTEN hamartoma tumor syndromeOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TNPO3Shared pathway100%RANBP6Shared pathway100%SPRNShared pathway100%FAM53AShared pathway100%RPAINShared pathway100%TMCO6Shared pathway100%
Tissue Expression6 tissues
Heart
100%
Brain
58%
Ovary
51%
Liver
48%
Lung
47%
Bone Marrow
32%
Gene Interaction Network
Click a node to explore
GPN3TNPO3RANBP6SPRNFAM53ARPAINTMCO6
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9UHW5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.81LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.16 [0.71–1.81]
RankingsWhere GPN3 stands among ~20K protein-coding genes
  • #8,430of 20,598
    Most Researched53
  • #16,630of 17,882
    Most Constrained (LOEUF)1.81
Genes detectedGPN3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
GTPase GPN3 facilitates cell proliferation and migration in non-small cell lung cancer by impeding clathrin-mediated endocytosis of EGFR.
PMID: 39893205
Cell Death Discov · 2025
1.00
2
Npa3 interacts with Gpn3 and assembly factor Rba50 for RNA polymerase II biogenesis.
PMID: 32985767
FASEB J · 2020
0.90
3
Gpn3 Is Essential for Cell Proliferation of Breast Cancer Cells Independent of Their Malignancy Degree.
PMID: 31431135
Technol Cancer Res Treat · 2019
0.80
4
Gpn3 is polyubiquitinated on lysine 216 and degraded by the proteasome in the cell nucleus in a Gpn1-inhibitable manner.
PMID: 29029378
FEBS Lett · 2017
0.70
5
Human GTPases associate with RNA polymerase II to mediate its nuclear import.
PMID: 21768307
Mol Cell Biol · 2011
0.60