RBBP9

Chr 20

RB binding protein 9, serine hydrolase

Also known as: BOG, RBBP10

The RBBP9 protein functions as a serine hydrolase and retinoblastoma binding protein that regulates cell proliferation, differentiation, and TGF-beta signaling pathways. Mutations cause autosomal recessive intellectual disability with seizures and variable dysmorphic features, typically presenting in early childhood. This gene shows tolerance to loss-of-function variants (pLI 0.05, LOEUF 0.81), consistent with recessive inheritance patterns.

Summary from RefSeq, UniProt
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0
Active trials
2
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.81
LOEUF
Mechanism
Clinical SummaryRBBP9
Population Constraint (gnomAD)
Low constraint (pLI 0.05) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.81LOEUF
pLI 0.049
Z-score 2.01
OE 0.35 (0.170.81)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.23Z-score
OE missense 0.94 (0.801.10)
100 obs / 106.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.35 (0.170.81)
00.351.4
Missense OE0.94 (0.801.10)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 4 / 11.3Missense obs/exp: 100 / 106.8Syn Z: 0.97

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RBBP9 · protein map & ClinVar variants

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

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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Key Publications
Landmark & review papers · by relevance
PubMed
Top 2 results · since 2015Search PubMed ↗