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GeneE
27 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HIPK2
homeodomain interacting protein kinase 2
Chromosome 7 · 7q34
NCBI Gene: 28996Ensembl: ENSG00000064393.17HGNC: HGNC:14402UniProt: Q9H2X6
228PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedKinaseTranscription Factor
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein kinase activitynegative regulation of ubiquitin-dependent protein catabolic processPML bodySMAD protein signal transductionhypertensionIncreased blood pressurecardiovascular diseaseneurodegenerative disease
✦AI Summary

HIPK2 is a serine/threonine protein kinase that functions primarily as a tumor suppressor through p53-dependent and -independent mechanisms. At the molecular level, HIPK2 phosphorylates p53 and multiple transcriptional regulators (including ATF1, CREB1, RUNX1, and EP300) to promote apoptosis and inhibit cell growth 1. HIPK2 acts as a transcriptional corepressor of pro-survival factors like CTBP1 and β-catenin, promoting their proteasomal degradation [UniProt]. The kinase is regulated primarily through post-translational mechanisms: caspase-dependent processing hyperactivates HIPK2, while ubiquitin-proteasome system-mediated degradation inactivates it, with both processes controlled by p53 2. In disease contexts, HIPK2 shows context-dependent roles. Exercise downregulates cardiac HIPK2, protecting against myocardial infarction by reducing p53-mediated cardiomyocyte apoptosis 3, suggesting HIPK2 suppression may be cardioprotective. Conversely, in cancer, HIPK2 typically functions as an oncosuppressor, and its downregulation correlates with chemoresistance and poor prognosis 4. However, HIPK2 overexpression in HPV-positive tonsillar carcinomas associates with worse prognosis, indicating tumor context-dependent functions 5. HIPK2 is also implicated in neurological disorders through stress response pathways and is highly expressed in nervous systems 6. These multifaceted roles make HIPK2 a potential therapeutic target and prognostic biomarker across multiple disease states.

Sources cited
1
Exercise downregulates cardiac HIPK2; HIPK2 inhibition protects against myocardial infarction through p53-dependent mechanisms
PMID: 34837851
2
HIPK2 and HIPK3 produce abundant circular RNAs from their second exon in humans and mice
PMID: 23249747
3
HIPK2 roles in cancer are context-dependent, ranging from tumor suppressive to oncogenic, with alterations including downregulation, upregulation, mutations, and delocalization
PMID: 36241057
4
HIPK2 is highly expressed in central and peripheral nervous systems; involved in endoplasmic reticulum stress response and neurological disorder pathogenesis
PMID: 36935052
5
HIPK2 activity is regulated post-translationally through caspase-dependent processing and ubiquitin-proteasome system degradation, both controlled by p53
PMID: 18974774
6
HIPK2 overexpression in HPV-positive tonsillar squamous cell carcinoma associates with poor prognosis and disease-free survival
PMID: 28607924
7
HIPK2 phosphorylates p53 for apoptotic activation; HIPK2 loss leads to chemoresistance and tumor growth, while HIPK2 overexpression induces apoptosis and chemosensitivity
PMID: 22889244
8
TGF-β signaling modulation discussed as therapeutic approach; HIPK2 cooperates with DAXX in TGF-β response pathways
PMID: 39990662
Disease Associationsⓘ20
hypertensionOpen Targets
0.49Moderate
Increased blood pressureOpen Targets
0.38Weak
cardiovascular diseaseOpen Targets
0.38Weak
neurodegenerative diseaseOpen Targets
0.35Weak
essential hypertensionOpen Targets
0.30Weak
thyroid diseaseOpen Targets
0.29Weak
hypothyroidismOpen Targets
0.29Weak
Hashimoto's thyroiditisOpen Targets
0.29Weak
response to xenobiotic stimulusOpen Targets
0.28Weak
autoimmune diseaseOpen Targets
0.28Weak
VitiligoOpen Targets
0.26Weak
Abnormality of the gastrointestinal tractOpen Targets
0.25Weak
liver diseaseOpen Targets
0.25Weak
muscular diseaseOpen Targets
0.25Weak
injuryOpen Targets
0.23Weak
generalized dystoniaOpen Targets
0.17Weak
neoplasmOpen Targets
0.11Weak
renal fibrosisOpen Targets
0.10Suggestive
non-small cell lung carcinomaOpen Targets
0.09Suggestive
SepsisOpen Targets
0.08Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
EP300Protein interaction100%DAXXProtein interaction100%SIAH1Protein interaction98%AXIN1Protein interaction98%PIN1Protein interaction97%PMLProtein interaction97%
Tissue Expression6 tissues
Brain
100%
Heart
67%
Liver
26%
Bone Marrow
22%
Lung
17%
Ovary
8%
Gene Interaction Network
Click a node to explore
HIPK2EP300DAXXSIAH1AXIN1PIN1PML
PROTEIN STRUCTURE
Preparing viewer…
PDB6P5S · 2.19 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.33Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.24 [0.17–0.33]
RankingsWhere HIPK2 stands among ~20K protein-coding genes
  • #1,770of 20,598
    Most Researched228 · top 10%
  • #1,405of 17,882
    Most Constrained (LOEUF)0.33 · top 10%
Genes detectedHIPK2
Sources retrieved27 papers
Response time—
📄 Sources
27▼
1
Exercise downregulates HIPK2 and HIPK2 inhibition protects against myocardial infarction.
PMID: 34837851
EBioMedicine · 2021
1.00
2
Spatial transcriptomics analysis identifies therapeutic targets in diffuse high-grade gliomas.
PMID: 39530009
Front Mol Neurosci · 2024
0.92
3
Circular RNAs are abundant, conserved, and associated with ALU repeats.
PMID: 23249747
RNA · 2013
0.90
4
Inactivation of HIPK2 attenuates KRAS
PMID: 39342278
J Exp Clin Cancer Res · 2024
0.88
5
Identification of Senomorphic miRNAs in Embryonic Progenitor and Adult Stem Cell-Derived Extracellular Vesicles.
PMID: 40275616
Aging Cell · 2025
0.84