ZBTB47

Chr 3

zinc finger and BTB domain containing 47

Also known as: ZNF651

ZBTB47 encodes a DNA-binding transcription factor that regulates gene transcription by RNA polymerase II in the nucleus. This gene is extremely intolerant to loss-of-function variants, but no specific Mendelian diseases have been definitively associated with ZBTB47 mutations in current clinical literature. The high constraint scores suggest that mutations in this gene may cause developmental or neurological phenotypes, but further research is needed to establish clear genotype-phenotype correlations.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.12
Clinical SummaryZBTB47
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
8 unique Pathogenic / Likely Pathogenic· 147 VUS of 184 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.12LOEUF
pLI 1.000
Z-score 4.61
OE 0.00 (0.000.12)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
2.45Z-score
OE missense 0.66 (0.600.73)
275 obs / 415.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.12)
00.351.4
Missense OE0.66 (0.600.73)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 0 / 24.8Missense obs/exp: 275 / 415.5Syn Z: 0.23
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedZBTB47-related developmental delay, intellectual disability, hypotonia and seizuresOTHERAD
DN
0.3693th %ile
GOF
0.3194th %ile
LOF
0.75top 10%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · LOEUF 0.12

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

184 submitted variants in ClinVar

Classification Summary

Pathogenic7
Likely Pathogenic1
VUS147
Likely Benign15
Benign1
Conflicting3
7
Pathogenic
1
Likely Pathogenic
147
VUS
15
Likely Benign
1
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
6
0
7
Likely Pathogenic
0
0
1
0
1
VUS
5
137
5
0
147
Likely Benign
0
9
1
5
15
Benign
0
0
0
1
1
Conflicting
3
Total5147136174

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ZBTB47 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found