ARHGEF28

Chr 5

Rho guanine nucleotide exchange factor 28

Also known as: RGNEF, RIP2, p190RHOGEF

This protein functions as a RHOA-specific guanine nucleotide exchange factor that regulates axonal branching, synapse formation, and dendritic morphogenesis. Mutations cause neurodevelopmental disorders with intellectual disability and developmental delay, following an autosomal dominant inheritance pattern. The gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to cause significant developmental impairment.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.70
Clinical SummaryARHGEF28
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Gene-Disease Validity (ClinGen)
amyotrophic lateral sclerosis · SDLimited

Limited evidence — not for standalone diagnostic reporting

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

Tolerant — LoF & missense variants common in population
LoF Constraint
0.70LOEUF
pLI 0.000
Z-score 3.83
OE 0.55 (0.440.70)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.73Z-score
OE missense 0.93 (0.880.99)
823 obs / 884.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.55 (0.440.70)
00.351.4
Missense OE0.93 (0.880.99)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 47 / 85.1Missense obs/exp: 823 / 884.4Syn Z: 1.61
DN
0.6356th %ile
GOF
0.6249th %ile
LOF
0.3842th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ARHGEF28 · 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