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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZHX3
zinc fingers and homeoboxes 3
Chromosome 20 · 20q12
NCBI Gene: 23051Ensembl: ENSG00000174306.23HGNC: HGNC:15935UniProt: Q9H4I2
57PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of DNA-templated transcriptionnucleusDNA-binding transcription factor activityprotein bindingAbnormality of the skeletal systemtype 2 diabetes mellitusandrogenetic alopeciadiabetes mellitus
✦AI Summary

ZHX3 is a ubiquitously expressed zinc finger and homeobox transcription factor that functions primarily as a transcriptional repressor 1. It contains two C2H2-type zinc finger motifs and five homeodomains, enabling heterodimerization with ZHX1 and ZHX2, as well as homodimerization 12. ZHX3 regulates multiple cellular processes through DNA binding at promoter regions and interaction with cofactors like CEBPB and NF-YA. Mechanistically, ZHX3 represses gluconeogenic genes (PCK1, G6PC1) in hepatocytes and suppresses uric acid transporter expression via CEBPB interaction, thereby regulating glucose metabolism and fasting blood glucose levels 3. In podocytes, nuclear localization of ZHX3 is regulated by heterodimerization status, with reduced nuclear sequestration associated with nephrotic syndrome development 4. ZHX3 loss induces cellular senescence through upregulation of senescence-associated genes like p16INK4a and ribosomal RNA genes 5. Clinically, ZHX3 demonstrates context-dependent roles: it promotes early mesenchymal stem cell osteogenic differentiation and serves as an osteogenic marker 6, yet functions as an oncogene in urothelial bladder carcinoma by repressing RGS2 and promoting cell migration and invasion 7. SNP associations with type 2 diabetes risk reflect ZHX3's metabolic regulatory function 3, positioning it as a therapeutic target in both metabolic and neoplastic diseases.

Sources cited
1
ZHX3 represses hepatic gluconeogenic genes PCK1 and G6PC1 via interaction with CEBPB, regulates uric acid secretion, and is associated with fasting blood glucose levels and T2D risk
PMID: 39990763
2
Loss of ZHX3 induces senescence-associated gene expression including p16INK4a and ribosomal RNA genes; ZHX3 is downregulated in replicative and oncogene-induced senescence
PMID: 35085309
3
ZHX3 regulates podocyte gene expression during minimal change disease; nuclear localization is controlled by heterodimerization with ZHX1 and ZHX2
PMID: 17056598
4
ZHX3 is involved in early MSC osteogenic differentiation and serves as an early osteogenic marker superior to RUNX2 or Osterix at initial differentiation stages
PMID: 21174497
5
ZHX3 is a ubiquitous transcriptional repressor containing two zinc-finger motifs and five homeodomains; forms dimers and interacts with NF-YA activation domain
PMID: 12659632
6
ZHX3 forms heterodimers with ZHX2 via homeodomains; ubiquitously expressed with multiple transcript sizes
PMID: 14659886
7
ZHX3 promotes urothelial bladder carcinoma progression by repressing RGS2; is a substrate of TRIM21-mediated ubiquitination and degradation
PMID: 33440047
8
ZHX family proteins (including ZHX3) function as double-edged swords in cancer, exhibiting both tumor-suppressive and oncogenic roles depending on context
PMID: 36232466
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.52Moderate
type 2 diabetes mellitusOpen Targets
0.46Moderate
androgenetic alopeciaOpen Targets
0.44Moderate
diabetes mellitusOpen Targets
0.42Moderate
HypercholesterolemiaOpen Targets
0.41Moderate
metabolic diseaseOpen Targets
0.33Weak
ovarian dysfunctionOpen Targets
0.32Weak
hyperlipidemiaOpen Targets
0.32Weak
ovarian neoplasmOpen Targets
0.31Weak
coronary artery diseaseOpen Targets
0.31Weak
cataractOpen Targets
0.31Weak
alopeciaOpen Targets
0.30Weak
alcohol drinkingOpen Targets
0.29Weak
skull disorderOpen Targets
0.29Weak
migraine disorderOpen Targets
0.29Weak
Abnormality of refractionOpen Targets
0.29Weak
pathological myopiaOpen Targets
0.29Weak
diverticular diseaseOpen Targets
0.27Weak
DNA methylationOpen Targets
0.25Weak
diabetic eye diseaseOpen Targets
0.24Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NFYAProtein interaction86%SUCOShared pathway33%SCUBE3Shared pathway33%TENT5AShared pathway33%DNAI3Shared pathway20%PRMT3Shared pathway20%
Tissue Expression6 tissues
Ovary
100%
Brain
91%
Heart
80%
Liver
66%
Lung
61%
Bone Marrow
30%
Gene Interaction Network
Click a node to explore
ZHX3NFYASUCOSCUBE3TENT5ADNAI3PRMT3
PROTEIN STRUCTURE
Preparing viewer…
PDB2DA5 · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.43Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.30 [0.21–0.43]
RankingsWhere ZHX3 stands among ~20K protein-coding genes
  • #8,033of 20,598
    Most Researched57
  • #2,318of 17,882
    Most Constrained (LOEUF)0.43 · top quartile
Genes detectedZHX3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
ZHX3 interacts with CEBPB to repress hepatic gluconeogenic gene expression and uric acid secretion.
PMID: 39990763
PNAS Nexus · 2025
1.00
2
Loss of the transcription repressor ZHX3 induces senescence-associated gene expression and mitochondrial-nucleolar activation.
PMID: 35085309
PLoS One · 2022
0.90
3
ZHX proteins regulate podocyte gene expression during the development of nephrotic syndrome.
PMID: 17056598
J Biol Chem · 2006
0.80
4
Impact of zinc fingers and homeoboxes 3 on the regulation of mesenchymal stem cell osteogenic differentiation.
PMID: 21174497
Stem Cells Dev · 2011
0.70
5
Zinc Fingers and Homeobox Family in Cancer: A Double-Edged Sword.
PMID: 36232466
Int J Mol Sci · 2022
0.60