RAB3GAP1

Chr 2AR

RAB3 GTPase activating protein catalytic subunit 1

Also known as: MARTS2, P130, RAB3GAP, RAB3GAP130, WARBM1

The protein functions as the catalytic subunit of a Rab3 GTPase-activating complex that regulates GTP hydrolysis by Rab proteins and has guanine nucleotide exchange factor activity toward RAB18, playing essential roles in eye and brain development. Mutations cause Warburg micro syndrome 1 and Martsolf syndrome 2, both autosomal recessive disorders affecting neurodevelopment with ocular abnormalities. The gene is highly constrained against loss-of-function variants (LOEUF 0.535), reflecting its critical developmental functions.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.542 OMIM phenotypes
Clinical SummaryRAB3GAP1
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Gene-Disease Validity (ClinGen)
Warburg micro syndrome · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.54LOEUF
pLI 0.000
Z-score 4.50
OE 0.38 (0.270.54)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.18Z-score
OE missense 0.85 (0.790.92)
440 obs / 515.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.38 (0.270.54)
00.351.4
Missense OE0.85 (0.790.92)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 23 / 60.9Missense obs/exp: 440 / 515.6Syn Z: 0.27

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RAB3GAP1 · 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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