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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RHOG
ras homolog family member G
Chromosome 11 · 11p15.4
NCBI Gene: 391Ensembl: ENSG00000177105.11HGNC: HGNC:672UniProt: P84095
113PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
Rho protein signal transductionRac protein signal transductionactin cytoskeleton organizationpositive regulation of DNA-templated transcriptionatrial fibrillationcancerkidney transplanteosinophilic esophagitis
✦AI Summary

RHOG (ras homolog family member G) is a small GTPase that functions as a critical regulator of cytoskeletal dynamics, lymphocyte cytotoxicity, and cell signaling. As a GTPase, RHOG cycles between inactive GDP-bound and active GTP-bound states, with its activation mediated by guanine nucleotide exchange factors (GEFs) such as Trio and the DOCK/ELMO complex 1. RHOG's primary mechanism involves orchestrating actin cytoskeleton reorganization and coordinating the activation of downstream GTPases, particularly Rac1, through interaction with DOCK5/ELMO1, where RhoG facilitates a conformational transition from closed to open states that enhances GEF activity 2. In lymphocytes, RHOG serves essential functions in cytotoxic granule exocytosis by maintaining granule docking at the plasma membrane through interaction with Munc13-4 3, and regulates gene expression including interferon-gamma and NFAT-dependent transcription 4. Clinically, RHOG deficiency causes hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory disorder characterized by severely impaired cytotoxic T cell and natural killer cell function 35. Conversely, RHOG knockout in CAR T cells unexpectedly enhances therapeutic efficacy, suggesting therapeutic potential for engineered cell-based immunotherapies 6. RHOG also participates in phagocytosis through Rac1-mediated pathways in trabecular meshwork cells 7.

Sources cited
1
RHOG knockout enhances CAR T cell efficacy and is a potent CAR T cell enhancer in immunotherapy
PMID: 40993398
2
RhoG deficiency causes hemophagocytic lymphohistiocytosis through impaired cytotoxic granule exocytosis; RhoG interacts with Munc13-4 for granule docking at plasma membrane
PMID: 33513601
3
RhoG facilitates conformational transition of DOCK5/ELMO1 complex from closed to open state, enhancing Rac GEF activity
PMID: 38857861
4
RhoG regulates interferon-gamma promoter and NFAT gene transcription in lymphocytes; promotes T-cell spreading and actin polymerization
PMID: 12545154
5
RhoG participates in Rac1-mediated phagocytosis through interaction with ELMO2 and ILK in trabecular meshwork cells
PMID: 27539661
6
Trio GEF activates RhoG to promote neurite outgrowth through NGF pathway
PMID: 11864571
7
RhoG deficiency identified as novel cause of primary hemophagocytic lymphohistiocytosis among inborn errors of immunity
PMID: 36843347
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.39Weak
cancerOpen Targets
0.37Weak
kidney transplantOpen Targets
0.29Weak
eosinophilic esophagitisOpen Targets
0.25Weak
genetic disorderOpen Targets
0.12Weak
cardiac arrhythmiaOpen Targets
0.10Suggestive
glioblastomaOpen Targets
0.08Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.08Suggestive
Chronic mucocutaneous candidosisOpen Targets
0.07Suggestive
immunodeficiency 86Open Targets
0.05Suggestive
immune deficiency, familial variableOpen Targets
0.05Suggestive
classic Hodgkin lymphomaOpen Targets
0.05Suggestive
Hodgkins lymphomaOpen Targets
0.05Suggestive
isolated agammaglobulinemiaOpen Targets
0.05Suggestive
atrial flutterOpen Targets
0.05Suggestive
inherited hemoglobinopathyOpen Targets
0.05Suggestive
hyper-IgM syndrome type 3Open Targets
0.05Suggestive
severe combined immunodeficiency due to CARD11 deficiencyOpen Targets
0.05Suggestive
multiple myelomaOpen Targets
0.05Suggestive
hyper-IgM syndrome type 2Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DOCK1Protein interaction100%DOCK2Protein interaction100%PIK3CBProtein interaction100%ARHGEF26Protein interaction97%VAV1Protein interaction95%ELMO1Protein interaction94%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
90%
Brain
39%
Ovary
28%
Liver
25%
Heart
18%
Gene Interaction Network
Click a node to explore
RHOGDOCK1DOCK2PIK3CBARHGEF26VAV1ELMO1
PROTEIN STRUCTURE
Preparing viewer…
PDB7Y4A · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.37Moderately Constrained
pLIⓘ
0.99Intolerant
Observed/Expected LoF0.08 [0.03–0.37]
RankingsWhere RHOG stands among ~20K protein-coding genes
  • #4,203of 20,598
    Most Researched113 · top quartile
  • #1,682of 17,882
    Most Constrained (LOEUF)0.37 · top 10%
Genes detectedRHOG
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Systematic discovery of CRISPR-boosted CAR T cell immunotherapies.
PMID: 40993398
Nature · 2025
1.00
2
RhoG deficiency abrogates cytotoxicity of human lymphocytes and causes hemophagocytic lymphohistiocytosis.
PMID: 33513601
Blood · 2021
0.90
3
RhoG facilitates a conformational transition in the guanine nucleotide exchange factor complex DOCK5/ELMO1 to an open state.
PMID: 38857861
J Biol Chem · 2024
0.80
4
RhoG regulates gene expression and the actin cytoskeleton in lymphocytes.
PMID: 12545154
Oncogene · 2003
0.70
5
Involvement of Tiam1, RhoG and ELMO2/ILK in Rac1-mediated phagocytosis in human trabecular meshwork cells.
PMID: 27539661
Exp Cell Res · 2016
0.60