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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZNF219
zinc finger protein 219
Chromosome 14 · 14q11.2
NCBI Gene: 51222Ensembl: ENSG00000165804.17HGNC: HGNC:13011UniProt: Q9P2Y4
40PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of DNA-templated transcriptionDNA bindingDNA-binding transcription factor activitynucleusneurodegenerative diseasealcohol drinkingstricturebreast disease
✦AI Summary

ZNF219 is a nuclear zinc finger transcription factor containing nine C2H2 zinc finger domains that recognizes the core DNA sequence motif 5'-CCCCC-3' 1. The protein functions as both a transcriptional repressor and activator depending on promoter context. ZNF219 represses HMGN1 expression 1 and prototype foamy virus replication through 5'LTR targeting 2. Conversely, ZNF219 activates chondrogenic gene expression by forming a transcriptional complex with SOX9 to enhance COL2A1, aggrecan, and COL11A2 expression during chondrocyte differentiation 3. Beyond skeletal development, ZNF219 is phosphorylated by CDKL5 in neurons and associates with the NuRD chr14 remodeling complex, regulating neuronal activity-dependent genes 4. ZNF219 also regulates SNCA expression in primary cortical neurons and represses neural differentiation in radial glia 5. Clinically, ZNF219 loss enhances anti-tumor immunity in aged T cells by inducing cytotoxic gene reprogramming, and high ZNF219 expression correlates with poorer survival following immune checkpoint blockade 6. Additionally, ZNF219 protects lens epithelial cells against oxidative stress via AKT/GSK3β pathway activation, suggesting roles in cataract pathogenesis 7. ZNF219 exhibits developmentally regulated transcript sizes and ubiquitous tissue expression 8.

Sources cited
1
ZNF219 recognizes core DNA sequence 5'-CCCCC-3', binds HMGN1 promoter, and functions as a transcriptional repressor
PMID: 14621294
2
ZNF219 represses prototype foamy virus replication by targeting viral 5'LTR promoter
PMID: 37356580
3
ZNF219 activates chondrogenic genes (COL2A1, aggrecan, COL11A2) by assembling transcription factory with SOX9
PMID: 20940257
4
ZNF219 is phosphorylated by CDKL5 in neurons and belongs to NuRD complex regulating neuronal genes
PMID: 39136782
5
ZNF219 represses neural differentiation in cortical radial glia during neurogenesis
PMID: 41565813
6
ZNF219 loss enhances anti-tumor immunity in aged T cells; high ZNF219 expression correlates with poor survival after immunotherapy
PMID: 41602280
7
ZNF219 protects lens epithelial cells from oxidative stress via AKT/GSK3β pathway activation
PMID: 34167360
8
ZNF219 contains 9 zinc finger domains, shows ubiquitous expression, developmentally regulated transcripts, and nuclear localization
PMID: 10819330
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.21Weak
alcohol drinkingOpen Targets
0.12Weak
strictureOpen Targets
0.11Weak
breast diseaseOpen Targets
0.10Weak
essential thrombocythemiaOpen Targets
0.08Suggestive
ThrombocytopeniaOpen Targets
0.07Suggestive
thrombocythemia 2Open Targets
0.06Suggestive
MODYOpen Targets
0.06Suggestive
macrothrombocytopenia, isolated, 2, autosomal dominantOpen Targets
0.06Suggestive
autosomal dominant macrothrombocytopeniaOpen Targets
0.05Suggestive
familial isolated congenital aspleniaOpen Targets
0.05Suggestive
thrombocytopenia 4Open Targets
0.05Suggestive
thrombocythemia 3Open Targets
0.05Suggestive
maturity-onset diabetes of the young type 3Open Targets
0.05Suggestive
Glycogen storage disease due to hepatic glycogen synthase deficiencyOpen Targets
0.05Suggestive
primary familial polycythemia due to EPO receptor mutationOpen Targets
0.05Suggestive
familial renal glucosuriaOpen Targets
0.05Suggestive
Renal glucosuriaOpen Targets
0.05Suggestive
Pyruvate kinase hyperactivityOpen Targets
0.05Suggestive
dicarboxylic aminoaciduriaOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GATAD2AProtein interaction100%RBBP4Protein interaction99%GATAD2BProtein interaction99%HDAC2Protein interaction98%MTA3Protein interaction98%RBBP7Protein interaction97%
Tissue Expression6 tissues
Lung
100%
Ovary
97%
Liver
57%
Brain
46%
Heart
31%
Bone Marrow
11%
Gene Interaction Network
Click a node to explore
ZNF219GATAD2ARBBP4GATAD2BHDAC2MTA3RBBP7
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9P2Y4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.77LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.51 [0.35–0.77]
RankingsWhere ZNF219 stands among ~20K protein-coding genes
  • #10,267of 20,598
    Most Researched40
  • #6,171of 17,882
    Most Constrained (LOEUF)0.77
Genes detectedZNF219
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Isolation and characterization of a novel zinc finger gene, ZNF219, and mapping to the human chromosome 14q11 region.
PMID: 10819330
DNA Res · 2000
1.00
2
Novel CDKL5 targets identified in human iPSC-derived neurons.
PMID: 39136782
Cell Mol Life Sci · 2024
0.90
3
Identification of the DNA binding specificity of the human ZNF219 protein and its function as a transcriptional repressor.
PMID: 14621294
DNA Res · 2003
0.80
4
CRISPR screens identify targets to rescue age-related T cell dysfunction in cancer.
PMID: 41602280
bioRxiv · 2026
0.70
5
Dissecting gene regulatory networks governing human cortical cell fate.
PMID: 41565813
Nature · 2026
0.60