BPTF

Chr 17

bromodomain PHD finger transcription factor

Also known as: FAC1, FALZ, NEDDFL, NURF301

The BPTF protein is the largest subunit of the NURF chromatin remodeling complex and catalyzes nucleosome sliding on DNA, which is essential for transcription regulation. Loss-of-function mutations cause autosomal dominant neurodevelopmental disorder with dysmorphic facies and distal limb anomalies. The gene is extremely intolerant to loss-of-function variants, with haploinsufficiency being the likely disease mechanism.

ResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismLOEUF 0.10
Clinical SummaryBPTF
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Gene-Disease Validity (ClinGen)
syndromic intellectual disability · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

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)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.10LOEUF
pLI 1.000
Z-score 10.12
OE 0.05 (0.030.10)
Highly constrained

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

Missense Constraint
3.71Z-score
OE missense 0.73 (0.690.77)
1067 obs / 1466.8 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.05 (0.030.10)
00.351.4
Missense OE0.73 (0.690.77)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 7 / 132.8Missense obs/exp: 1067 / 1466.8Syn Z: -0.48
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongBPTF-related developmental and speech delay, postnatal microcephaly, and dysmorphic featuresLOFAD
DN
0.17100th %ile
GOF
0.15100th %ile
LOF
0.88top 5%

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

LOFprediction above median · 1 literature citation · LOEUF 0.10

Literature Evidence

LOFHaploinsufficiency of the chromatin remodeler BPTF causes syndromic developmental and speech delay, postnatal microcephaly, and dysmorphic features.PMID:28942966

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

BPTF · 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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