DAB2IP

Chr 9

DAB2 interacting protein

Also known as: AF9Q34, AIP-1, AIP1, DIP1/2

DAB2IP encodes a Ras GTPase-activating protein that functions as a scaffold protein regulating multiple signaling pathways including cell cycle control, apoptosis, inflammation, and neuronal migration, and acts as a tumor suppressor. Loss-of-function mutations cause autosomal recessive intellectual disability and developmental delay, with the protein being critical for cortical neuron migration and cerebellar synapse formation during brain development. The gene is highly intolerant to loss-of-function variants, supporting a haploinsufficiency mechanism of disease.

OMIMResearchSummary from RefSeq, UniProt, Mechanism
LOFmechanismLOEUF 0.20
Clinical SummaryDAB2IP
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.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.20LOEUF
pLI 1.000
Z-score 5.72
OE 0.09 (0.040.20)
Highly constrained

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

Missense Constraint
2.36Z-score
OE missense 0.75 (0.700.81)
522 obs / 697.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.09 (0.040.20)
00.351.4
Missense OE0.75 (0.700.81)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 4 / 45.7Missense obs/exp: 522 / 697.5Syn Z: -1.06
DN
0.4190th %ile
GOF
0.4974th %ile
LOF
0.75top 10%

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

LOFprediction above median · LOEUF 0.20

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

DAB2IP · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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