BAIAP2

Chr 17AD

BAR/IMD domain containing adaptor protein 2

Also known as: BAP2, DEE120, FLAF3, IRSP53, WAML

The protein functions as an adapter that links membrane-bound small G-proteins to cytoplasmic effector proteins, regulating actin cytoskeleton reorganization and promoting neurite growth and filopodia formation. Mutations cause developmental and epileptic encephalopathy 120, inherited in an autosomal dominant pattern. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.43), and the encephalopathy presents in early development with both developmental delays and epilepsy.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismADLOEUF 0.431 OMIM phenotype
Clinical SummaryBAIAP2
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.22) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.43LOEUF
pLI 0.462
Z-score 3.81
OE 0.22 (0.120.43)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
2.06Z-score
OE missense 0.69 (0.630.77)
249 obs / 358.8 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.22 (0.120.43)
00.351.4
Missense OE0.69 (0.630.77)
00.61.4
Synonymous OE1.34
01.21.6
LoF obs/exp: 6 / 27.6Missense obs/exp: 249 / 358.8Syn Z: -3.41
DN
0.6745th %ile
GOF
0.6150th %ile
LOF
0.55top 25%

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BAIAP2 · 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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