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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ARHGAP9
Rho GTPase activating protein 9
Chromosome 12 · 12q13.3
NCBI Gene: 64333Ensembl: ENSG00000123329.21HGNC: HGNC:14130UniProt: B3KQ74
30PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
GTPase activator activityprotein bindingphosphatidylinositol-3,4,5-trisphosphate bindingcytoplasmautosomal recessive spastic paraplegia type 70central nervous system infectionautoimmune diseasesevere early-onset pulmonary alveolar proteinosis due to MARS deficiency
✦AI Summary

ARHGAP9 (Rho GTPase activating protein 9) functions as a GTPase activator that converts active GTP-bound Rho family GTPases to their inactive GDP-bound state, with substantial GAP activity toward CDC42 and RAC1, and lesser activity toward RHOA 1. The protein contains multiple functional domains including a Rho-GAP domain, SH3 domain, pleckstrin homology region, and WW domain 1. ARHGAP9 regulates cellular adhesion of hematopoietic cells to extracellular matrix components like fibronectin and collagen IV 1. Mechanistically, ARHGAP9 acts as a MAP kinase docking protein that inhibits ERK and p38 activation through WW domain binding, representing a novel cross-talk mechanism between Rho GTPase and MAP kinase signaling 2. The protein also regulates cancer progression through multiple pathways, including PI3K/AKT/mTOR signaling inhibition 3 and Wnt/β-catenin pathway modulation via DKK2 suppression 4. ARHGAP9 expression is predominantly found in peripheral blood leukocytes, spleen, and thymus 1. Clinical significance varies by cancer type: ARHGAP9 overexpression correlates with poor prognosis in acute myeloid leukemia 5 and clear cell renal cell carcinoma 6, while downregulation is associated with poor outcomes in hepatocellular carcinoma 7 and colorectal cancer 3.

Sources cited
1
ARHGAP9 has GAP activity toward CDC42, RAC1, and RHOA; contains multiple functional domains; regulates hematopoietic cell adhesion
PMID: 11396949
2
ARHGAP9 acts as a MAP kinase docking protein that inhibits ERK and p38 activation through WW domain binding
PMID: 17284314
3
ARHGAP9 inhibits PI3K/AKT/mTOR signaling pathway in colorectal cancer
PMID: 35679685
4
ARHGAP9 modulates Wnt/β-catenin pathway via DKK2 suppression in lung adenocarcinoma
PMID: 37454937
5
ARHGAP9 overexpression correlates with poor prognosis in acute myeloid leukemia
PMID: 33579308
6
ARHGAP9 upregulation correlates with poor prognosis in clear cell renal cell carcinoma
PMID: 39465715
7
ARHGAP9 downregulation is associated with poor outcomes in hepatocellular carcinoma
PMID: 30206221
Disease Associationsⓘ20
autosomal recessive spastic paraplegia type 70Open Targets
0.33Weak
central nervous system infectionOpen Targets
0.27Weak
autoimmune diseaseOpen Targets
0.22Weak
Charcot-Marie-Tooth disease axonal type 2UOpen Targets
0.19Weak
severe early-onset pulmonary alveolar proteinosis due to MARS deficiencyOpen Targets
0.19Weak
hereditary spastic paraplegiaOpen Targets
0.12Weak
acute myeloid leukemiaOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.08Suggestive
lung adenocarcinomaOpen Targets
0.07Suggestive
injuryOpen Targets
0.07Suggestive
breast cancerOpen Targets
0.06Suggestive
DNA methylationOpen Targets
0.04Suggestive
cancerOpen Targets
0.04Suggestive
ovarian serous adenocarcinomaOpen Targets
0.03Suggestive
neoplasmOpen Targets
0.03Suggestive
goutOpen Targets
0.03Suggestive
liver cancerOpen Targets
0.03Suggestive
colorectal carcinomaOpen Targets
0.03Suggestive
polycystic ovary syndromeOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ARHGAP31Shared pathway100%CDC42Protein interaction84%PLEKProtein interaction80%TBC1D10CProtein interaction79%RAC2Protein interaction73%PLEK2Protein interaction71%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
39%
Liver
10%
Brain
3%
Heart
2%
Ovary
2%
Gene Interaction Network
Click a node to explore
ARHGAP9ARHGAP31CDC42PLEKTBC1D10CRAC2PLEK2
PROTEIN STRUCTURE
Preparing viewer…
PDB2P0D · 1.81 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.02LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.70–1.02]
RankingsWhere ARHGAP9 stands among ~20K protein-coding genes
  • #11,853of 20,598
    Most Researched30
  • #10,080of 17,882
    Most Constrained (LOEUF)1.02
Genes detectedARHGAP9
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Isolation of a novel human gene, ARHGAP9, encoding a rho-GTPase activating protein.
PMID: 11396949
Biochem Biophys Res Commun · 2001
1.00
2
Rho GTPase Activating Protein 9 (ARHGAP9) in Human Cancers.
PMID: 34365932
Recent Pat Anticancer Drug Discov · 2022
0.90
3
The role of ARHGAP9: clinical implication and potential function in acute myeloid leukemia.
PMID: 33579308
J Transl Med · 2021
0.80
4
ARHGAP9 suppresses the migration and invasion of hepatocellular carcinoma cells through up-regulating FOXJ2/E-cadherin.
PMID: 30206221
Cell Death Dis · 2018
0.70
5
ARHGAP9 knockdown promotes lung adenocarcinoma metastasis by activating Wnt/β-catenin signaling pathway via suppressing DKK2.
PMID: 37454937
Genomics · 2023
0.60