AEBP2 encodes an accessory subunit of the Polycomb repressive complex 2 (PRC2) that mediates histone H3K27 trimethylation and plays a role in nucleosome localization of the PRC2 complex for transcriptional repression. Mutations cause neurodevelopmental disorders with intellectual disability, developmental delay, and dysmorphic features, typically with onset in early childhood. This gene shows autosomal dominant inheritance and is highly constrained against loss-of-function variants.

Summary from RefSeq, UniProt
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0
Active trials
3
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.14
LOEUF· LoF intol.
LOF
Mechanism· predicted
Clinical SummaryAEBP2
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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.14LOEUF
pLI 0.999
Z-score 4.21
OE 0.00 (0.000.14)
Highly constrained

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

Missense Constraint
2.21Z-score
OE missense 0.59 (0.510.68)
135 obs / 229.4 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.14)
00.351.4
Missense OE0.59 (0.510.68)
00.61.4
Synonymous OE1.17
01.21.6
LoF obs/exp: 0 / 20.7Missense obs/exp: 135 / 229.4Syn Z: -1.19
DN
0.2399th %ile
GOF
0.1999th %ile
LOF
0.88top 5%

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

LOFprediction above median · LOEUF 0.14

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

AEBP2 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC