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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HIC2
HIC ZBTB transcriptional repressor 2
Chromosome 22 · 22q11.21
NCBI Gene: 23119Ensembl: ENSG00000169635.11HGNC: HGNC:18595UniProt: Q96JB3
54PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingnucleusnucleoplasmregulation of cytokine productionneurodegenerative diseasegliomaleft ventricular noncompactionHeterotaxia
✦AI Summary

HIC2 (HIC ZBTB transcriptional repressor 2) is a zinc finger transcriptional regulator with dual roles in developmental and disease contexts. Primary function: HIC2 acts as a transcriptional repressor that controls developmental hemoglobin switching by repressing BCL11A transcription in erythroid cells 1. During fetal development, HIC2 is highly expressed and inhibits BCL11A through direct binding to erythroid enhancers, reducing chr22 accessibility and GATA1 binding 1. This HIC2-mediated repression is developmentally downregulated by let-7 miRNAs in adult cells, allowing BCL11A reactivation and fetal hemoglobin silencing 2. Mechanism: HIC2 functions through direct DNA binding and protein-protein interactions. In hemoglobin switching, it causes steric hindrance of transcription factor GATA1 binding 1. Notably, HIC2 exhibits opposing functions to its homolog HIC1: while HIC1 represses SIRT1, HIC2 activates SIRT1 transcription, potentially protecting cardiac tissue from ischemia-reperfusion injury 3. Disease relevance: HIC2 functions as a tumor suppressor in glioma and glioblastoma. It is hypermethylated and downregulated in these malignancies, predicting poor prognosis 4. HIC2 suppresses glioblastoma progression by transcriptionally repressing SEMA3A and attenuating TGF-β signaling 5. HIC2 also participates in bladder cancer chemoresistance regulation through miRNA-mediated mechanisms 6. Clinical significance: HIC2 represents a therapeutic target for hemoglobinopathies and brain tumors, with potential applications in personalized cancer therapy and cardiac protection.

Sources cited
1
HIC2 represses BCL11A transcription to control fetal-to-adult hemoglobin switching through enhancer binding and GATA1 inhibition
PMID: 35941187
2
Let-7 miRNAs directly repress HIC2 expression, establishing the let-7→HIC2→BCL11A→HBG regulatory axis in hemoglobin development
PMID: 38364109
3
HIC2 is hypermethylated and downregulated in glioma, functioning as an independent prognostic factor and tumor suppressor through RNF44 and PTPRN2 interactions
PMID: 36650953
4
HIC2 suppresses glioblastoma progression by transcriptionally repressing SEMA3A and inhibiting TGF-β/Smad signaling
PMID: 40921301
5
Unlike HIC1, HIC2 is a transcriptional activator of SIRT1 and may protect cardiomyocytes from ischemia-reperfusion injury
PMID: 31127867
6
HIC2 is a ZBTB family member with roles in cardiac development and disease regulation
PMID: 40398093
7
HIC2 is a target of miR-193a-3p and participates in bladder cancer chemoresistance through multiple signaling pathways
PMID: 25188512
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.51Moderate
gliomaOpen Targets
0.07Suggestive
left ventricular noncompactionOpen Targets
0.06Suggestive
HeterotaxiaOpen Targets
0.05Suggestive
Aortic CoarctationOpen Targets
0.05Suggestive
atrioventricular septal defect 5Open Targets
0.04Suggestive
congenital heart diseaseOpen Targets
0.04Suggestive
congenital heart defects, multiple types, 4Open Targets
0.04Suggestive
congenital left-sided heart lesionsOpen Targets
0.04Suggestive
atrial septal defect 1Open Targets
0.04Suggestive
atrial heart septal defectOpen Targets
0.04Suggestive
ventricular septal defect 1Open Targets
0.04Suggestive
tricuspid atresiaOpen Targets
0.04Suggestive
22q11.2 deletion syndromeOpen Targets
0.04Suggestive
congenital unilateral hypoplasia of depressor anguli orisOpen Targets
0.04Suggestive
Fallot complex - intellectual disability - growth delayOpen Targets
0.04Suggestive
fallot complex-intellectual disability-growth delay syndromeOpen Targets
0.04Suggestive
congenital heart defects, multiple types, 6Open Targets
0.04Suggestive
Congenitally uncorrected transposition of the great arteriesOpen Targets
0.04Suggestive
congenital heart defects, multiple types, 5Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ZBTB14Shared pathway100%ZBTB25Shared pathway100%ZNF131Shared pathway100%ZNF134Shared pathway100%ZBTB39Shared pathway100%ZBTB6Shared pathway100%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
52%
Liver
28%
Lung
26%
Heart
15%
Brain
13%
Gene Interaction Network
Click a node to explore
HIC2ZBTB14ZBTB25ZNF131ZNF134ZBTB39ZBTB6
PROTEIN STRUCTURE
Preparing viewer…
PDB7TXC · 3.04 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.20Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.06 [0.03–0.20]
RankingsWhere HIC2 stands among ~20K protein-coding genes
  • #8,322of 20,598
    Most Researched54
  • #468of 17,882
    Most Constrained (LOEUF)0.20 · top 5%
Genes detectedHIC2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
HIC2 controls developmental hemoglobin switching by repressing BCL11A transcription.
PMID: 35941187
Nat Genet · 2022
1.00
2
let-7 miRNAs repress HIC2 to regulate BCL11A transcription and hemoglobin switching.
PMID: 38364109
Blood · 2024
0.90
3
Hypermethylation of HIC2 is a potential prognostic biomarker and tumor suppressor of glioma based on bioinformatics analysis and experiments.
PMID: 36650953
CNS Neurosci Ther · 2023
0.80
4
HIC2 suppresses glioblastoma progression via transcriptional repression of SEMA3A and inhibition of TGF-β signaling.
PMID: 40921301
Free Radic Biol Med · 2025
0.70
5
HIC2, a new transcription activator of SIRT1.
PMID: 31127867
FEBS Lett · 2019
0.60