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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ARHGEF28
Rho guanine nucleotide exchange factor 28
Chromosome 5 · 5q13.2
NCBI Gene: 64283Ensembl: ENSG00000214944.11HGNC: HGNC:30322UniProt: Q8N1W1
46PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
guanyl-nucleotide exchange factor activitysmall GTPase-mediated signal transductionregulation of Rho protein signal transductionregulation of small GTPase mediated signal transductionhearing lossage-related hearing impairmentIntrahepatic cholestasis of pregnancyHodgkins lymphoma
✦AI Summary

ARHGEF28 encodes a Rho guanine nucleotide exchange factor (RGNEF) that functions as a RHOA-specific GEF regulating signaling downstream of integrins and growth factor receptors. The protein mediates axonal branching, synapse formation, dendritic morphogenesis, focal adhesion formation, and cell motility. ARHGEF28 acts as an NFL mRNA destabilizing factor, binding and affecting neurofilament mRNA stability 1. In amyotrophic lateral sclerosis (ALS), rare coding variants in ARHGEF28 show marginal enrichment in sporadic ALS cases, with the p.Asn1046Ser variant significantly more frequent in patients 2. Frameshift and truncation mutations have been identified in both familial and sporadic ALS cohorts 13. Beyond ALS, ARHGEF28 variants are associated with familial non-medullary thyroid cancer through predicted protein structure alterations and protein-protein interactions involving TP53 and FOXE1 pathways 4. ARHGEF28 also emerges as a prognostic RNA-binding protein in esophageal cancer prognosis models 5 and shows diagnostic potential in psoriasis, where expression is significantly decreased compared to normal skin 6. Recent evidence links ARHGEF28 to alpha-synucleinopathies through RhoA/actin cytoskeleton regulation mechanisms shared with Alzheimer's disease pathways 7. Common variant associations with age-related hearing loss have also been reported 8.

Sources cited
1
Rare coding variants in ARHGEF28 show marginal enrichment in sporadic ALS cases; p.Asn1046Ser variant more frequent in patients; low-frequency and common variants not associated with ALS
PMID: 31060816
2
RGNEF (ARHGEF28 protein product) is an NFL mRNA destabilizing factor; frameshift mutations identified in familial ALS cases; regions of homozygosity observed in ARHGEF28
PMID: 23286752
3
Intron 6 junction mutations in ARHGEF28 gene identified in sporadic ALS patients with altered clinical features
PMID: 24712971
4
ARHGEF28 associated with familial non-medullary thyroid cancer through protein structure alterations; high thyroid expression; protein interactions with SQSTM1, TP53, PTCSC2, and FOXE1
PMID: 37175943
5
ARHGEF28 identified as prognostic RNA-binding protein in esophageal cancer prognostic risk model with predictive ability for 5-year survival
PMID: 39287291
6
ARHGEF28 identified as diagnostic gene for psoriasis with significantly decreased expression in psoriatic lesions; expressed in mast cells
PMID: 38803495
7
ARHGEF28 is an RhoA/actin cytoskeleton regulator shared as risk factor across alpha-synucleinopathies; rare variants associated with Parkinson's disease, Lewy body dementia, and multiple system atrophy
PMID: 38496508
8
ARHGEF28 common variant associations identified with age-related hearing loss etiology
PMID: 36788145
Disease Associationsⓘ20
hearing lossOpen Targets
0.51Moderate
age-related hearing impairmentOpen Targets
0.46Moderate
Intrahepatic cholestasis of pregnancyOpen Targets
0.41Moderate
Hodgkins lymphomaOpen Targets
0.34Weak
cholestasis, intrahepatic, of pregnancy 3Open Targets
0.33Weak
Sensorineural hearing impairmentOpen Targets
0.32Weak
liver diseaseOpen Targets
0.31Weak
disorder of earOpen Targets
0.30Weak
sensorineural hearing lossOpen Targets
0.30Weak
schizophreniaOpen Targets
0.25Weak
KeloidOpen Targets
0.24Weak
VitiligoOpen Targets
0.24Weak
multinodular goiterOpen Targets
0.23Weak
prostate carcinomaOpen Targets
0.21Weak
amyotrophic lateral sclerosisOpen Targets
0.20Weak
HemoptysisOpen Targets
0.17Weak
Meniere diseaseOpen Targets
0.16Weak
polycythemia veraOpen Targets
0.14Weak
goutOpen Targets
0.14Weak
diaphragm diseaseOpen Targets
0.12Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PTK2Protein interaction94%PTK2BProtein interaction93%MCF2Protein interaction93%RHOAProtein interaction92%RHOCProtein interaction81%NEFMProtein interaction77%
Tissue Expression6 tissues
Ovary
100%
Liver
27%
Brain
15%
Lung
13%
Heart
8%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
ARHGEF28PTK2PTK2BMCF2RHOARHOCNEFM
PROTEIN STRUCTURE
Preparing viewer…
PDB6BC0 · 2.20 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.90LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.77 [0.67–0.90]
RankingsWhere ARHGEF28 stands among ~20K protein-coding genes
  • #9,302of 20,598
    Most Researched46
  • #8,053of 17,882
    Most Constrained (LOEUF)0.90
Genes detectedARHGEF28
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Rare, low-frequency and common coding variants of ARHGEF28 gene and their association with sporadic amyotrophic lateral sclerosis.
PMID: 31060816
Neurobiol Aging · 2020
1.00
2
Detection of a novel frameshift mutation and regions with homozygosis within ARHGEF28 gene in familial amyotrophic lateral sclerosis.
PMID: 23286752
Amyotroph Lateral Scler Frontotemporal Degener · 2013
0.90
3
Construction and validation of a prognostic model for esophageal cancer based on prognostic-related RNA-binding protein.
PMID: 39287291
Medicine (Baltimore) · 2024
0.80
4
Discovery of biomarkers in the psoriasis through machine learning and dynamic immune infiltration in three types of skin lesions.
PMID: 38803495
Front Immunol · 2024
0.70
5
Novel Susceptibility Genes Drive Familial Non-Medullary Thyroid Cancer in a Large Consanguineous Kindred.
PMID: 37175943
Int J Mol Sci · 2023
0.60