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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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WIZ
WIZ zinc finger
Chromosome 19 · 19p13.12
NCBI Gene: 58525Ensembl: ENSG00000011451.21HGNC: HGNC:30917UniProt: A0A2R8YFV2
111PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
extracellular exosomeDNA-binding transcription factor activity, RNA polymerase II-specifictranscription corepressor bindingprotein bindingneurodegenerative diseasebladder exstrophyankyloblepharon filiforme adnatum-cleft palate syndromeAnkyloblepharon filiforme adnatum - cleft palate
✦AI Summary

WIZ (widely interspaced zinc finger-containing protein) is a transcriptional regulator that functions through multiple molecular mechanisms to control gene expression and chr19 architecture. Primarily, WIZ serves as a component of the G9a/GLP histone methyltransferase complex, where it targets these enzymes to specific chr19 loci via its zinc finger DNA-binding domains to mediate H3K9 methylation and transcriptional repression 1. Beyond this repressive function, WIZ also participates in DNA loop architecture by forming a complex with cohesin and CTCF at enhancers, promoters, and loop anchors, where it is required for proper transcriptional regulation and maintenance of cell identity 2. ChIP-seq analysis reveals WIZ occupancy at active promoters and CTCF-binding sites, suggesting a dual role as both repressor and activator; notably, WIZ is highly expressed in brain tissue and regulates clustered protocadherin genes essential for neuronal development 3. Clinically, WIZ has emerged as a therapeutic target in sickle cell disease: molecular glue degraders that recruit WIZ to cereblon for proteasomal degradation robustly induce fetal hemoglobin expression in erythroid cells and show therapeutic efficacy in preclinical models 45. Additionally, WIZ mutations correlate with prognosis in oral squamous cell carcinoma, suggesting potential disease relevance beyond hemoglobinopathies 6.

Sources cited
1
WIZ targets the G9a/GLP histone methyltransferase complex to chromatin via zinc finger-mediated DNA binding, mediating H3K9 methylation and transcriptional repression
PMID: 25789554
2
WIZ forms a complex with cohesin and CTCF that anchors DNA loops and regulates gene expression and cell identity
PMID: 32294452
3
WIZ binds active promoters and CTCF sites in brain, regulates protocadherin genes, and is required for normal behavior in mice
PMID: 27410475
4
WIZ degradation via molecular glue degraders induces fetal hemoglobin expression as a therapeutic strategy for sickle cell disease
PMID: 38963839
5
Optimized WIZ molecular glue degraders demonstrate robust HbF induction in preclinical models of sickle cell disease
PMID: 39541509
6
WIZ mutations correlate with prognostic markers and pathological stage in oral squamous cell carcinoma
PMID: 36586077
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.38Weak
bladder exstrophyOpen Targets
0.12Weak
Ankyloblepharon filiforme adnatum - cleft palateOpen Targets
0.05Suggestive
ankyloblepharon filiforme adnatum-cleft palate syndromeOpen Targets
0.05Suggestive
Hypoplastic amelogenesis imperfectaOpen Targets
0.05Suggestive
amelogenesis imperfectaOpen Targets
0.04Suggestive
OligodontiaOpen Targets
0.04Suggestive
tooth agenesisOpen Targets
0.04Suggestive
anencephaly 2Open Targets
0.04Suggestive
Choanal atresia-deafness-cardiac defects-dysmorphism syndromeOpen Targets
0.04Suggestive
Ankyloblepharon filiforme - imperforate anusOpen Targets
0.04Suggestive
ankyloblepharon filiforme-imperforate anus syndromeOpen Targets
0.04Suggestive
dentin dysplasia type IOpen Targets
0.04Suggestive
orofacial cleft 15Open Targets
0.04Suggestive
amelogenesis imperfecta, type ijOpen Targets
0.03Suggestive
Pai syndromeOpen Targets
0.03Suggestive
frontofacionasal dysplasiaOpen Targets
0.03Suggestive
Cleft palate - short stature - vertebral anomaliesOpen Targets
0.03Suggestive
cleft palate-short stature-vertebral anomalies syndromeOpen Targets
0.03Suggestive
cleft palate-lateral synechia syndromeOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CBX1Protein interaction94%CTBP1Protein interaction85%CDYLProtein interaction82%ZNF644Protein interaction82%EHMT1Protein interaction78%EHMT2Protein interaction73%
Tissue Expression6 tissues
Ovary
100%
Liver
86%
Lung
82%
Heart
53%
Bone Marrow
49%
Brain
49%
Gene Interaction Network
Click a node to explore
WIZCBX1CTBP1CDYLZNF644EHMT1EHMT2
PROTEIN STRUCTURE
Preparing viewer…
PDB9DJT · 2.95 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.29Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.21 [0.15–0.29]
RankingsWhere WIZ stands among ~20K protein-coding genes
  • #4,305of 20,598
    Most Researched111 · top quartile
  • #1,065of 17,882
    Most Constrained (LOEUF)0.29 · top 10%
Genes detectedWIZ
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A molecular glue degrader of the WIZ transcription factor for fetal hemoglobin induction.
PMID: 38963839
Science · 2024
1.00
2
A WIZ/Cohesin/CTCF Complex Anchors DNA Loops to Define Gene Expression and Cell Identity.
PMID: 32294452
Cell Rep · 2020
0.90
3
The zinc finger proteins ZNF644 and WIZ regulate the G9a/GLP complex for gene repression.
PMID: 25789554
Elife · 2015
0.80
4
Discovery and Optimization of First-in-Class Molecular Glue Degraders of the WIZ Transcription Factor for Fetal Hemoglobin Induction to Treat Sickle Cell Disease.
PMID: 39541509
J Med Chem · 2024
0.70
5
Wiz binds active promoters and CTCF-binding sites and is required for normal behaviour in the mouse.
PMID: 27410475
Elife · 2016
0.60