| Name | RHO family interacting cell polarization regulator 2 |
| Description | This gene encodes an atypical inhibitor of the small G protein RhoA. Inhibition of RhoA activity by the encoded protein mediates myoblast fusion and polarization of T cells and neutrophils. The encoded protein is a component of hair cell stereocilia that is essential for hearing. A splice site mutation in this gene results in hearing loss in human patients. [provided by RefSeq, Sep 2016] |
| Summary |
{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nRIPOR2 (also known as FAM65B) plays an essential role in the function of sensory hair cells in the inner ear. In these cells, RIPOR2 localizes to the plasma membrane of stereocilia where it assembles into oligomeric ring‐like structures critical for establishing proper cell architecture and mechanotransduction. Disruption of RIPOR2 localization—through mutation or misprocessing—leads to defective stereocilia structure and is associated with congenital, prelingual, profound hearing loss."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nBeyond its role in auditory function, RIPOR2 influences cell differentiation and cytoskeletal organization in multiple tissues. In muscle, RIPOR2 is a critical component of a complex with HDAC6 and dysferlin, and its proper expression is required for myogenic differentiation and myoblast fusion. In the immune system, RIPOR2 functions as an atypical inhibitor of the small G protein RhoA; upon phosphorylation, its dissociation from RhoA promotes cytoskeletal rearrangements that are vital for optimal T lymphocyte migration and neutrophil polarization. Furthermore, genome‐wide neuroimaging studies have linked FAM65B to variations in cortical thickness and white matter integrity, suggesting that RIPOR2 may also contribute to neuronal migration and brain structural organization."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "3", "end_ref": "6"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nIn the context of oncogenesis, particularly in cervical cancer, RIPOR2 expression is markedly reduced in cells expressing high‐risk human papillomavirus oncoproteins E6 and E7. This downregulation correlates with progression of premalignant lesions and is strongly associated with adverse overall survival, implicating RIPOR2 as a potential tumor suppressor."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": "\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Oscar Diaz-Horta, Asli Subasioglu-Uzak, M'hamed Grati, et al. "}, {"type": "b", "children": [{"type": "t", "text": "FAM65B is a membrane-associated protein of hair cell stereocilia required for hearing."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Proc Natl Acad Sci U S A (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1073/pnas.1401950111"}], "href": "https://doi.org/10.1073/pnas.1401950111"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24958875"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24958875"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Bo Zhao, Zizhen Wu, Ulrich Müller "}, {"type": "b", "children": [{"type": "t", "text": "Murine Fam65b forms ring-like structures at the base of stereocilia critical for mechanosensory hair cell function."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.14222"}], "href": "https://doi.org/10.7554/eLife.14222"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "27269051"}], "href": "https://pubmed.ncbi.nlm.nih.gov/27269051"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Anuradha Balasubramanian, Genri Kawahara, Vandana A Gupta, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Fam65b is important for formation of the HDAC6-dysferlin protein complex during myogenic cell differentiation."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "FASEB J (2014)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1096/fj.13-246470"}], "href": "https://doi.org/10.1096/fj.13-246470"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "24687993"}], "href": "https://pubmed.ncbi.nlm.nih.gov/24687993"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Kun Gao, Wenwen Tang, Yuan Li, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Front-signal-dependent accumulation of the RHOA inhibitor FAM65B at leading edges polarizes neutrophils."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.161497"}], "href": "https://doi.org/10.1242/jcs.161497"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25588844"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25588844"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Laura Megrelis, Elyas El Ghoul, Federica Moalli, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Fam65b Phosphorylation Relieves Tonic RhoA Inhibition During T Cell Migration."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Front Immunol (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3389/fimmu.2018.02001"}], "href": "https://doi.org/10.3389/fimmu.2018.02001"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30254631"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30254631"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "John D Eicher, Angela M Montgomery, Natacha Akshoomoff, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Dyslexia and language impairment associated genetic markers influence cortical thickness and white matter in typically developing children."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Brain Imaging Behav (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s11682-015-9392-6"}], "href": "https://doi.org/10.1007/s11682-015-9392-6"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25953057"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25953057"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Leslie Olmedo-Nieva, J Omar Muñoz-Bello, Imelda Martínez-Ramírez, et al. "}, {"type": "b", "children": [{"type": "t", "text": "RIPOR2 Expression Decreased by HPV-16 E6 and E7 Oncoproteins: An Opportunity in the Search for Prognostic Biomarkers in Cervical Cancer."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cells (2022)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.3390/cells11233942"}], "href": "https://doi.org/10.3390/cells11233942"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "36497200"}], "href": "https://pubmed.ncbi.nlm.nih.gov/36497200"}]}]}]}
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| NCBI Gene ID | 9750 |
| API | |
| Download Associations | |
| Predicted Functions |
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| Co-expressed Genes |
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| Expression in Tissues and Cell Lines |
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RIPOR2 has 3,028 functional associations with biological entities spanning 6 categories (chemical, disease, phenotype or trait, functional term, phrase or reference, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 39 datasets.
Click the + buttons to view associations for RIPOR2 from the datasets below.
If available, associations are ranked by standardized value
| Dataset | Summary | |
|---|---|---|
| Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles | tissue samples with high or low expression of RIPOR2 gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset. | |
| Carcinogenome Chemical Perturbation Carcinogenicity Signatures | small molecule perturbations changing expression of RIPOR2 gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset. | |
| CellMarker Gene-Cell Type Associations | cell types associated with RIPOR2 gene from the CellMarker Gene-Cell Type Associations dataset. | |
| ChEA Transcription Factor Targets 2022 | transcription factors binding the promoter of RIPOR2 gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset. | |
| ClinVar Gene-Phenotype Associations 2025 | phenotypes associated with RIPOR2 gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset. | |
| CM4AI KOLF21J CRISPRi Gene Perturbation Atlas | gene perturbations changing expression of RIPOR2 gene from the CM4AI KOLF21J CRISPRi Gene Perturbation Atlas dataset. | |
| COMPARTMENTS Curated Protein Localization Evidence Scores 2025 | cellular components containing RIPOR2 protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset. | |
| COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 | cellular components co-occuring with RIPOR2 protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset. | |
| DepMap CRISPR Gene Dependency | cell lines with fitness changed by RIPOR2 gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset. | |
| DGIdb Drug Targets 2026 | interacting drugs for RIPOR2 protein from the DGIdb Drug Targets 2026 dataset. | |
| DISEASES Curated Gene-Disease Association Evidence Scores 2025 | diseases involving RIPOR2 gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset. | |
| DISEASES Text-mining Gene-Disease Association Evidence Scores 2025 | diseases co-occuring with RIPOR2 gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset. | |
| DisGeNET Gene-Disease Associations | diseases associated with RIPOR2 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset. | |
| DisGeNET Gene-Phenotype Associations | phenotypes associated with RIPOR2 gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset. | |
| GO Biological Process Annotations 2023 | biological processes involving RIPOR2 gene from the curated GO Biological Process Annotations 2023 dataset. | |
| GO Biological Process Annotations 2025 | biological processes involving RIPOR2 gene from the curated GO Biological Process Annotations2025 dataset. | |
| GO Cellular Component Annotations 2023 | cellular components containing RIPOR2 protein from the curated GO Cellular Component Annotations 2023 dataset. | |
| GO Cellular Component Annotations 2025 | cellular components containing RIPOR2 protein from the curated GO Cellular Component Annotations 2025 dataset. | |
| GTEx eQTL 2025 | SNPs regulating expression of RIPOR2 gene from the GTEx eQTL 2025 dataset. | |
| GTEx Tissue Gene Expression Profiles 2023 | tissues with high or low expression of RIPOR2 gene relative to other tissues from the GTEx Tissue Gene Expression Profiles 2023 dataset. | |
| GWAS Catalog SNP-Phenotype Associations 2025 | phenotypes associated with RIPOR2 gene in GWAS datasets from the GWAS Catalog SNP-Phenotype Associations 2025 dataset. | |
| HuBMAP Azimuth Cell Type Annotations | cell types associated with RIPOR2 gene from the HuBMAP Azimuth Cell Type Annotations dataset. | |
| JASPAR Predicted Human Transcription Factor Targets 2025 | transcription factors regulating expression of RIPOR2 gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Human Transcription Factor Targets dataset. | |
| JASPAR Predicted Mouse Transcription Factor Targets 2025 | transcription factors regulating expression of RIPOR2 gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Mouse Transcription Factor Targets 2025 dataset. | |
| LINCS L1000 CMAP Chemical Perturbation Consensus Signatures | small molecule perturbations changing expression of RIPOR2 gene from the LINCS L1000 CMAP Chemical Perturbations Consensus Signatures dataset. | |
| LINCS L1000 CMAP CRISPR Knockout Consensus Signatures | gene perturbations changing expression of RIPOR2 gene from the LINCS L1000 CMAP CRISPR Knockout Consensus Signatures dataset. | |
| MGI Mouse Phenotype Associations 2023 | phenotypes of transgenic mice caused by RIPOR2 gene mutations from the MGI Mouse Phenotype Associations 2023 dataset. | |
| MoTrPAC Rat Endurance Exercise Training | tissue samples with high or low expression of RIPOR2 gene relative to other tissue samples from the MoTrPAC Rat Endurance Exercise Training dataset. | |
| NIBR DRUG-seq U2OS MoA Box Gene Expression Profiles | drug perturbations changing expression of RIPOR2 gene from the NIBR DRUG-seq U2OS MoA Box dataset. | |
| PFOCR Pathway Figure Associations 2023 | pathways involving RIPOR2 protein from the PFOCR Pathway Figure Associations 2023 dataset. | |
| PFOCR Pathway Figure Associations 2024 | pathways involving RIPOR2 protein from the Wikipathways PFOCR 2024 dataset. | |
| Reactome Pathways 2024 | pathways involving RIPOR2 protein from the Reactome Pathways 2024 dataset. | |
| Rummagene Transcription Factor Associations 2026 | transcription factors regulating expression of RIPOR2 gene from the Rummagene Transcription Factor Associations 2026 dataset. | |
| RummaGEO Drug Perturbation Signatures | drug perturbations changing expression of RIPOR2 gene from the RummaGEO Drug Perturbation Signatures dataset. | |
| RummaGEO Gene Perturbation Signatures | gene perturbations changing expression of RIPOR2 gene from the RummaGEO Gene Perturbation Signatures dataset. | |
| Tahoe Therapeutics Tahoe 100M Perturbation Atlas | drug perturbations changing expression of RIPOR2 gene from the Tahoe Therapeutics Tahoe 100M Perturbation Atlas dataset. | |
| TISSUES Curated Tissue Protein Expression Evidence Scores 2025 | tissues with high expression of RIPOR2 protein from the TISSUES Curated Tissue Protein Expression Evidence Scores 2025 dataset. | |
| TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 | tissues with high expression of RIPOR2 protein in proteomics datasets from the TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 dataset. | |
| TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 | tissues co-occuring with RIPOR2 protein in abstracts of biomedical publications from the TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 dataset. | |
