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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HIPK3
homeodomain interacting protein kinase 3
Chromosome 11 · 11p13
NCBI Gene: 10114Ensembl: ENSG00000110422.13HGNC: HGNC:4915UniProt: A8KAE4
68PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
KinaseTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein kinase activityprotein serine/threonine kinase activityprotein phosphorylationmRNA transcriptiongastrointestinal diseaseglaucomaneuroinflammatory disordercholangiocarcinoma
✦AI Summary

HIPK3 is a serine/threonine protein kinase that functions as a transcriptional regulator with pleiotropic roles in cellular signaling. As a kinase, HIPK3 phosphorylates key transcription factors including JUN and RUNX2, modulating their transcriptional activity 1. In steroidogenesis, HIPK3 phosphorylates NR5A1 to activate SF1, promoting steroidogenic gene expression via cAMP signaling [UniProt]. HIPK3 negatively regulates apoptosis by phosphorylating FADD and suppressing JUN kinase activity, thereby controlling cell survival pathways [UniProt]. Beyond the linear protein product, HIPK3 generates abundant circular RNA (circHIPK3) with independent regulatory functions 2. CircHIPK3 acts as a competitive endogenous RNA, sponging microRNAs to regulate cellular proliferation and viability 1. Dysregulation of HIPK3 is implicated in multiple human diseases including cancers, cardiovascular disease, diabetes mellitus, and kidney injury/regeneration 31. HIPK3 protein stability is regulated by WWP1-mediated ubiquitination and degradation, representing a critical post-translational control mechanism in cancer progression 4. In kidney regeneration, SOX9 modulates HIPK3 expression in tubular epithelial cells, suggesting involvement in injury response 5.

Sources cited
1
HIPKs including HIPK3 are Ser/Thr kinases involved in signal transduction, apoptosis, and multiple diseases; circRNAs from HIPK3 loci modulate proliferation by sponging miRNAs
PMID: 29793420
2
HIPK3 produces abundant circular RNA from its second exon in humans and mice; circular RNAs are stable and may act as competing endogenous RNAs
PMID: 23249747
3
CircHIPK3 has significant impacts on multiple human diseases including cancers, cardiovascular diseases, diabetes mellitus, and inflammatory diseases
PMID: 36292157
4
WWP1 mediates ubiquitination and degradation of HIPK3 protein; HIPK3 acts as a tumor suppressor; the WWP1/HIPK3 axis regulates JNK signaling in cancer
PMID: 40280416
5
SOX9 alters HIPK3 expression in kidney tubular epithelial cells during injury and regeneration
PMID: 36454702
Disease Associationsⓘ20
gastrointestinal diseaseOpen Targets
0.32Weak
glaucomaOpen Targets
0.25Weak
neuroinflammatory disorderOpen Targets
0.16Weak
cholangiocarcinomaOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.08Suggestive
esophageal squamous cell carcinomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.07Suggestive
hepatocellular carcinomaOpen Targets
0.07Suggestive
rheumatoid arthritisOpen Targets
0.06Suggestive
Huntington diseaseOpen Targets
0.06Suggestive
chronic pancreatitisOpen Targets
0.06Suggestive
hyperinsulinemic hypoglycemia, familial, 2Open Targets
0.05Suggestive
breast cancerOpen Targets
0.05Suggestive
colorectal cancerOpen Targets
0.05Suggestive
diabetes mellitusOpen Targets
0.05Suggestive
exercise-induced hyperinsulinismOpen Targets
0.05Suggestive
Partial pancreatic agenesisOpen Targets
0.05Suggestive
diabetic ketoacidosisOpen Targets
0.05Suggestive
GCGR-related hyperglucagonemiaOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RASSF5Protein interaction91%FADDProtein interaction82%SRSF5Protein interaction79%HNRNPH1Protein interaction77%TRA2BProtein interaction75%DCAF7Protein interaction71%
Tissue Expression6 tissues
Heart
100%
Lung
43%
Brain
36%
Ovary
36%
Bone Marrow
29%
Liver
26%
Gene Interaction Network
Click a node to explore
HIPK3RASSF5FADDSRSF5HNRNPH1TRA2BDCAF7
PROTEIN STRUCTURE
Preparing viewer…
PDB7O7I · 2.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.47Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.35 [0.27–0.47]
RankingsWhere HIPK3 stands among ~20K protein-coding genes
  • #6,904of 20,598
    Most Researched68
  • #2,655of 17,882
    Most Constrained (LOEUF)0.47 · top quartile
Genes detectedHIPK3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Circular RNAs are abundant, conserved, and associated with ALU repeats.
PMID: 23249747
RNA · 2013
1.00
2
The Diagnostic and Therapeutic Role of Circular RNA HIPK3 in Human Diseases.
PMID: 36292157
Diagnostics (Basel) · 2022
0.90
3
Short intronic repeat sequences facilitate circular RNA production.
PMID: 25281217
Genes Dev · 2014
0.80
4
Update on the Regulation of HIPK1, HIPK2 and HIPK3 Protein Kinases by microRNAs.
PMID: 29793420
Microrna · 2018
0.70
5
The circular RNA HIPK3 (circHIPK3) and its regulation in cancer progression: Review.
PMID: 31901445
Life Sci · 2020
0.60