GDI1

Chr XXLD

GDP dissociation inhibitor 1

GDP dissociation inhibitor 1 regulates the GDP/GTP exchange reaction of Rab proteins, which are essential for vesicular trafficking between cellular organelles. Mutations cause X-linked intellectual developmental disorder, with the gene being highly constrained against loss-of-function variants (pLI 0.99, LOEUF 0.19). The protein is expressed primarily in neural and sensory tissues and follows X-linked inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismXLDLOEUF 0.191 OMIM phenotype
Clinical SummaryGDI1
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Gene-Disease Validity (ClinGen)
non-syndromic X-linked intellectual disability · XLModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.99). One damaged copy is likely sufficient to cause disease.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.19LOEUF
pLI 0.995
Z-score 3.65
OE 0.00 (0.000.19)
Highly constrained

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

Missense Constraint
3.29Z-score
OE missense 0.35 (0.290.42)
70 obs / 201.4 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.00 (0.000.19)
00.351.4
Missense OE0.35 (0.290.42)
00.61.4
Synonymous OE1.24
01.21.6
LoF obs/exp: 0 / 15.6Missense obs/exp: 70 / 201.4Syn Z: -1.73
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedGDI1-related intellectual developmental disorderLOFXLR
DN
0.2499th %ile
GOF
0.4282th %ile
LOF
0.71top 10%

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

LOFprediction above median · LOEUF 0.19

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

GDI1 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC