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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ZBTB40
zinc finger and BTB domain containing 40
Chromosome 1 · 1p36.12
NCBI Gene: 9923Ensembl: ENSG00000184677.18HGNC: HGNC:29045UniProt: B3KR52
39PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
DNA damage responseprotein bindingnucleusDNA-binding transcription factor activity, RNA polymerase II-specificosteoporosisulcerative colitisDupuytren Contractureinflammatory bowel disease
✦AI Summary

ZBTB40 is a zinc finger and BTB domain-containing transcription factor with multifaceted roles in telomere biology, bone metabolism, and inflammatory disease. Primary function: ZBTB40 functions as a telomere-associated protein that binds telomeric dsDNA through its BTB/POZ domain to protect telomeres in alternative lengthening of telomeres (ALT) cells 1. ZBTB40 knockout results in telomere dysfunction and excessive lengthening, indicating it negatively regulates ALT-mediated telomere extension 1. Mechanism: In osteoblasts, ZBTB40 directly promotes bone mineralization and osteoblast activity 2. The protein antagonizes the suppressive effects of the long noncoding RNA ZBTB40-IT1 on osteogenesis, with ZBTB40-IT1 inhibiting bone formation via the miR-514a-3p/FOXO4 axis 3. Disease relevance: ZBTB40 genetic variants associate with reduced bone mineral density and osteoporosis susceptibility in both European and Han Chinese populations 4. Loss of ZBTB40 causes male infertility through impaired meiotic DNA repair, compromised double-strand break repair, and sperm morphology defects 5. ZBTB40 variants also increase inflammatory bowel disease (IBD) risk while reducing bone density, suggesting shared genetic mechanisms between these diseases 6. In myeloid cells, ZBTB40 regulates calprotectin expression, connecting it to IBD pathogenesis 7. Clinical significance: ZBTB40 represents a target for treating osteoporosis and male infertility, and may inform IBD-related bone loss management.

Sources cited
1
ZBTB40 is a telomere-associated protein that binds telomeric dsDNA and protects telomeres in ALT cells; ZBTB40 knockout causes telomere dysfunction and lengthening
PMID: 37454741
2
ZBTB40 loss causes male infertility with longer telomeres, compromised DSB repair, sperm flagellum deformities, and abnormal acrosome biogenesis; ZBTB40 mutations associate with non-obstructive azoospermia
PMID: 37174664
3
ZBTB40 risk variants associate with both increased IBD risk and reduced bone mineral density, indicating shared genetic underpinnings
PMID: 36680554
4
ZBTB40 protein promotes osteogenesis while lncRNA ZBTB40-IT1 suppresses it; osteoporosis GWAS SNPs regulate both ZBTB40 and ZBTB40-IT1 expression
PMID: 30661131
5
ZBTB40 SNPs associate with bone mineral density at lumbar spine and total hip in Han Chinese women, replicating European findings
PMID: 20554715
6
ZBTB40 knockdown in osteoblasts drastically reduces mineralization; loss-of-function models exhibit reduced bone mineral density
PMID: 32497039
7
ZBTB40-IT1 suppresses osteogenic differentiation via the miR-514a-3p/FOXO4 axis; ZBTB40-IT1 inhibition promotes bone formation
PMID: 35676609
8
ZBTB40 is among IBD GWAS loci genes that regulate calprotectin (S100A8/A9) expression in myeloid cells, linking it to IBD pathogenesis
PMID: 36155972
Disease Associationsⓘ20
osteoporosisOpen Targets
0.34Weak
ulcerative colitisOpen Targets
0.33Weak
Dupuytren ContractureOpen Targets
0.32Weak
inflammatory bowel diseaseOpen Targets
0.30Weak
bone diseaseOpen Targets
0.29Weak
Palmar FibromatosisOpen Targets
0.27Weak
bone fractureOpen Targets
0.27Weak
Alzheimer diseaseOpen Targets
0.26Weak
lysosomal storage diseaseOpen Targets
0.26Weak
multiple sclerosisOpen Targets
0.26Weak
neurodegenerative diseaseOpen Targets
0.26Weak
Parkinson diseaseOpen Targets
0.26Weak
Abnormality of the skeletal systemOpen Targets
0.24Weak
joint diseaseOpen Targets
0.23Weak
lumbar disc herniationOpen Targets
0.23Weak
mixed connective tissue diseaseOpen Targets
0.22Weak
ovarian dysfunctionOpen Targets
0.22Weak
placental retentionOpen Targets
0.21Weak
Crohn's diseaseOpen Targets
0.21Weak
intestinal diseaseOpen Targets
0.21Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MEGF6Protein interaction83%WRAP73Protein interaction83%PHOSPHO1Shared pathway50%SPINDOCShared pathway50%FLYWCH1Shared pathway50%DCPH1Shared pathway50%
Tissue Expression6 tissues
Ovary
100%
Lung
88%
Bone Marrow
79%
Heart
63%
Liver
54%
Brain
45%
Gene Interaction Network
Click a node to explore
ZBTB40MEGF6WRAP73PHOSPHO1SPINDOCFLYWCH1DCPH1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q1RMZ5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.60LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.49 [0.39–0.60]
RankingsWhere ZBTB40 stands among ~20K protein-coding genes
  • #10,421of 20,598
    Most Researched39
  • #4,152of 17,882
    Most Constrained (LOEUF)0.60 · top quartile
Genes detectedZBTB40
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
ZBTB40 is a telomere-associated protein and protects telomeres in human ALT cells.
PMID: 37454741
J Biol Chem · 2023
1.00
2
PMID: 37174664
Cells · 2023
0.90
3
Risk Variants in or Near ZBTB40 AND NFATC1 Increase the Risk of Both IBD and Adverse Bone Health Outcomes Highlighting Common Genetic Underpinnings Across Both Diseases.
PMID: 36680554
Inflamm Bowel Dis · 2023
0.80
4
LncRNA ZBTB40-IT1 modulated by osteoporosis GWAS risk SNPs suppresses osteogenesis.
PMID: 30661131
Hum Genet · 2019
0.70
5
Analysis of recently identified osteoporosis susceptibility genes in Han Chinese women.
PMID: 20554715
J Clin Endocrinol Metab · 2010
0.60