ARHGDIA

Chr 17

Rho GDP dissociation inhibitor alpha

Also known as: GDIA1, HEL-S-47e, NPHS8, RHOGDI, RHOGDI-1

This gene encodes a protein that plays a key role in the regulation of signaling through Rho GTPases. The encoded protein inhibits the disassociation of Rho family members from GDP (guanine diphosphate), thereby maintaining these factors in an inactive state. Activity of this protein is important in a variety of cellular processes, and expression of this gene may be altered in tumors. Mutations in this gene have been found in individuals with nephrotic syndrome, type 8. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jul 2014]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtNephrotic syndrome 8

Clinical highlights

Gene-disease validity (ClinGen)
nephrotic syndrome, type 8 · ARModerateconsider for supplementary testing
0
Active trials
5
Pubs (1 yr)
P/LP submissions
P/LP missense
0.87
LOEUF
DN
Mechanism· predicted
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.87LOEUF
pLI 0.036
Z-score 1.86
OE 0.38 (0.190.87)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.20Z-score
OE missense 0.70 (0.580.83)
87 obs / 124.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.38 (0.190.87)
00.351.4
Missense OE?0.70 (0.580.83)
00.61.4
Synonymous OE?1.57
01.21.6
LoF obs/exp: 4 / 10.5Missense obs/exp: 87 / 124.9Syn Z: -3.35

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ARHGDIA · 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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