RAPH1

Chr 2

Ras association (RalGDS/AF-6) and pleckstrin homology domains 1

Also known as: ALS2CR18, ALS2CR9, LPD, PREL-2, PREL2, RMO1, RalGDS/AF-6

The protein regulates actin dynamics and lamellipodial formation through interaction with Ena/VASP proteins and direct binding to filamentous actin, mediating localized membrane signals and cell adhesion. Mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability and seizures. This gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.22
Clinical SummaryRAPH1
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
0.22LOEUF
pLI 1.000
Z-score 5.69
OE 0.11 (0.050.22)
Highly constrained

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

Missense Constraint
1.39Z-score
OE missense 0.85 (0.790.91)
583 obs / 685.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.11 (0.050.22)
00.351.4
Missense OE0.85 (0.790.91)
00.61.4
Synonymous OE1.07
01.21.6
LoF obs/exp: 5 / 47.2Missense obs/exp: 583 / 685.1Syn Z: -0.94
DN
0.4289th %ile
GOF
0.3689th %ile
LOF
0.76top 10%

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

LOFprediction above median · LOEUF 0.22

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

RAPH1 · 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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