RALGAPA2

Chr 20

Ral GTPase activating protein catalytic subunit alpha 2

Also known as: AS250, C20orf74, bA287B20.1, dJ1049G11, dJ1049G11.4, p220

The RALGAPA2 protein serves as the catalytic subunit of the RalGAP2 complex, which activates GTPase activity for the small GTPases RALA and RALB involved in cellular signaling and protein localization. Mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability and developmental delay. This gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely pathogenic.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
25
P/LP submissions
0%
P/LP missense
0.59
LOEUF
DN
Mechanism· predicted
Clinical SummaryRALGAPA2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
25 unique Pathogenic / Likely Pathogenic· 286 VUS of 369 total submissions
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.59LOEUF
pLI 0.000
Z-score 4.98
OE 0.46 (0.360.59)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.89Z-score
OE missense 0.92 (0.870.97)
877 obs / 954.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.46 (0.360.59)
00.351.4
Missense OE0.92 (0.870.97)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 45 / 98.3Missense obs/exp: 877 / 954.2Syn Z: -0.68
DN
0.6161th %ile
GOF
0.5660th %ile
LOF
0.4136th %ile

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

369 submitted variants in ClinVar

Classification Summary

Pathogenic22
Likely Pathogenic3
VUS286
Likely Benign12
Benign2
22
Pathogenic
3
Likely Pathogenic
286
VUS
12
Likely Benign
2
Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
22
0
22
Likely Pathogenic
0
0
3
0
3
VUS
1
282
3
0
286
Likely Benign
0
9
0
3
12
Benign
0
1
0
1
2
Total1292284325

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

RALGAPA2 · 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

No open access results found