RBBP7

Chr XX-linked

RB binding protein 7, chromatin remodeling factor

Also known as: RbAp46, SPGFX9

The RBBP7 protein is a core histone-binding subunit that functions in multiple chromatin remodeling complexes, including histone acetyltransferase, histone deacetylase, and nucleosome remodeling complexes that regulate gene expression and chromatin assembly. Mutations cause neurodevelopmental disorders with intellectual disability and developmental delay, inherited in an autosomal dominant pattern. The gene is highly constrained against loss-of-function variants, indicating that even single functional copies are critical for normal development.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismX-linkedLOEUF 0.281 OMIM phenotype
Clinical SummaryRBBP7
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.28LOEUF
pLI 0.984
Z-score 3.59
OE 0.06 (0.020.28)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
2.79Z-score
OE missense 0.41 (0.340.50)
72 obs / 176.2 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.06 (0.020.28)
00.351.4
Missense OE0.41 (0.340.50)
00.61.4
Synonymous OE1.13
01.21.6
LoF obs/exp: 1 / 17.0Missense obs/exp: 72 / 176.2Syn Z: -0.80
DN
0.2499th %ile
GOF
0.2397th %ile
LOF
0.82top 5%

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

LOFprediction above median · LOEUF 0.28

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

RBBP7 · 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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