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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZNF207
zinc finger protein 207
Chromosome 17 · 17q11.2
NCBI Gene: 7756Ensembl: ENSG00000010244.20HGNC: HGNC:12998UniProt: O43670
100PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA bindingprotein bindingmicrotubule bindingmitotic sister chromatid segregationneurodegenerative diseaseAlzheimer diseasemultiple sclerosislysosomal storage disease
✦AI Summary

ZNF207 is a multifunctional zinc finger protein with roles spanning cell division, stem cell biology, and cancer progression. Canonically, ZNF207 functions as a kinetochore- and microtubule-binding protein essential for spindle assembly 12. ZNF207 contains low-complexity disordered regions that undergo phase transition to form liquid droplets, concentrating tubulin and promoting microtubule polymerization and spindle matrix assembly 3. It stabilizes the checkpoint protein BUB3 at kinetochores, though the precise mechanism remains unclear 12. Beyond mitosis, a distinct ZNF207 isoform regulates self-renewal and pluripotency in human embryonic stem cells by partnering with OCT4, SOX2, and NANOG, while simultaneously controlling neuronal differentiation 4. In cancer, ZNF207 exhibits oncogenic properties across multiple tumor types, particularly hepatocellular carcinoma (HCC). It promotes HCC progression by transcriptionally activating glycolytic enzymes ENO1 and GAPDH to enhance aerobic glycolysis 5, and drives regorafenib resistance through ZNF207-mediated PRDX1 lactylation and NRF2 activation, suppressing ferroptosis 6. ZNF207 also functions as an immunosuppressive factor, reducing CD8+ T-cell infiltration and promoting exhaustion via MAPK-chemokine signaling and IDO1-mediated kynurenine production 7. Recent evidence reveals unexpected involvement in LMNA splicing regulation, where ZNF207 interacts with U1 snRNP components to modulate alternative splicing, including progerin production in progeria 8. ZNF207 emerges as a pan-cancer prognostic biomarker and therapeutic target 910.

Sources cited
1
ZNF207 contains low-complexity disordered regions that undergo phase transition to form liquid droplets, concentrating tubulin and promoting microtubule polymerization and spindle matrix assembly .
PMID: 26388440
2
Beyond mitosis, a distinct ZNF207 isoform regulates self-renewal and pluripotency in human embryonic stem cells by partnering with OCT4, SOX2, and NANOG, while simultaneously controlling neuronal differentiation .
PMID: 30349051
3
It promotes HCC progression by transcriptionally activating glycolytic enzymes ENO1 and GAPDH to enhance aerobic glycolysis , and drives regorafenib resistance through ZNF207-mediated PRDX1 lactylation and NRF2 activation, suppressing ferroptosis .
PMID: 40684964
4
It promotes HCC progression by transcriptionally activating glycolytic enzymes ENO1 and GAPDH to enhance aerobic glycolysis , and drives regorafenib resistance through ZNF207-mediated PRDX1 lactylation and NRF2 activation, suppressing ferroptosis .
PMID: 40680452
5
ZNF207 also functions as an immunosuppressive factor, reducing CD8+ T-cell infiltration and promoting exhaustion via MAPK-chemokine signaling and IDO1-mediated kynurenine production .
PMID: 35246476
6
Recent evidence reveals unexpected involvement in LMNA splicing regulation, where ZNF207 interacts with U1 snRNP components to modulate alternative splicing, including progerin production in progeria .
PMID: 41475346
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.56Moderate
Alzheimer diseaseOpen Targets
0.46Moderate
lysosomal storage diseaseOpen Targets
0.46Moderate
multiple sclerosisOpen Targets
0.46Moderate
Parkinson diseaseOpen Targets
0.46Moderate
rectosigmoid junction neoplasmOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.09Suggestive
neoplasmOpen Targets
0.08Suggestive
Blackfan-Diamond anemiaOpen Targets
0.07Suggestive
smoking initiationOpen Targets
0.07Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.05Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.05Suggestive
schizophreniaOpen Targets
0.05Suggestive
Adult-onset autosomal recessive sideroblastic anemiaOpen Targets
0.05Suggestive
dehydrated hereditary stomatocytosisOpen Targets
0.05Suggestive
X-linked sideroblastic anemia 1Open Targets
0.04Suggestive
hemoglobin D diseaseOpen Targets
0.04Suggestive
IRIDA syndromeOpen Targets
0.04Suggestive
hemolytic anemia due to erythrocyte adenosine deaminase overproductionOpen Targets
0.04Suggestive
amegakaryocytic thrombocytopenia, congenital, 2Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SF3A3Protein interaction100%HNRNPMProtein interaction100%SRSF3Protein interaction73%FUBP1Protein interaction73%HNRNPUL1Protein interaction72%BUB3Protein interaction71%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
49%
Lung
46%
Brain
36%
Liver
33%
Heart
26%
Gene Interaction Network
Click a node to explore
ZNF207SF3A3HNRNPMSRSF3FUBP1HNRNPUL1BUB3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt O43670
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.60Moderately Constrained
pLIⓘ
0.13Tolerant
Observed/Expected LoF0.42 [0.31–0.60]
RankingsWhere ZNF207 stands among ~20K protein-coding genes
  • #4,822of 20,598
    Most Researched100 · top quartile
  • #4,077of 17,882
    Most Constrained (LOEUF)0.60 · top quartile
Genes detectedZNF207
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
ZNF207-driven PRDX1 lactylation and NRF2 activation in regorafenib resistance and ferroptosis evasion.
PMID: 40680452
Drug Resist Updat · 2025
1.00
2
System analysis based on the cancer-immunity cycle identifies ZNF207 as a novel immunotherapy target for hepatocellular carcinoma.
PMID: 35246476
J Immunother Cancer · 2022
0.90
3
A distinct isoform of ZNF207 controls self-renewal and pluripotency of human embryonic stem cells.
PMID: 30349051
Nat Commun · 2018
0.80
4
Transcription factor ZNF207 drives aerobic glycolysis and facilitates malignancy progression in hepatocellular carcinoma.
PMID: 40684964
Cell Signal · 2025
0.70
5
Gender-specific lncRNA-miRNA-mRNA regulatory network to reveal potential genes for primary open-angle glaucoma.
PMID: 37774963
Exp Eye Res · 2023
0.60