BAZ1B

Chr 7

bromodomain adjacent to zinc finger domain 1B

This gene encodes a chromatin remodeling protein that regulates transcription and DNA repair through ATP-dependent nucleosome repositioning and histone phosphorylation. BAZ1B is deleted in Williams-Beuren syndrome, a multisystem developmental disorder affecting cardiovascular, neurodevelopmental, and behavioral domains that typically presents in early childhood. The gene is highly constrained against loss-of-function variants in the general population.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.11
Clinical SummaryBAZ1B
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.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.11LOEUF
pLI 1.000
Z-score 7.48
OE 0.04 (0.020.11)
Highly constrained

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

Missense Constraint
3.72Z-score
OE missense 0.63 (0.590.68)
508 obs / 805.3 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.04 (0.020.11)
00.351.4
Missense OE0.63 (0.590.68)
00.61.4
Synonymous OE1.02
01.21.6
LoF obs/exp: 3 / 71.0Missense obs/exp: 508 / 805.3Syn Z: -0.32
DN
0.2199th %ile
GOF
0.1999th %ile
LOF
0.77top 5%

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

LOFprediction above median · LOEUF 0.11

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BAZ1B · 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.

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Clinical Literature
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