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    GENE:

    RREB1 (Ras Responsive Element Binding Protein 1)

    i
    Other names: RREB1, Ras Responsive Element Binding Protein 1, HNT, Zinc Finger Motif Enhancer-Binding Protein 1, Ras-Responsive Element-Binding Protein 1, Raf-Responsive Zinc Finger Protein LZ321, Finger Protein In Nuclear Bodies, RREB-1, Zep-1, FINB, Hindsight Homolog (Drosophila), DNA-Binding Protein, Hindsight Homolog, LZ321
    Associations

    News

    Trials
    2ms
    Cutaneous/subcutaneous RREB1::MRTFB fusion-positive extra-glossal mesenchymal neoplasm-two cases expanding the anatomical spectrum of an emerging entity. (PubMed, Virchows Arch)
    RREB1::MRTFB fusion was confirmed in both cases. In summary, the two reported cases expand the anatomical spectrum and improve our understanding of this rare emerging entity.
    Journal
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    RREB1 (Ras Responsive Element Binding Protein 1)
    7ms
    Ras-Responsive Element Binding Protein 1 regulates survival of Group 3 medulloblastoma. (PubMed, bioRxiv)
    Local delivery of SU11274 in tumor-bearing mice suppressed RREB1 expression and extended survival. These results establish the c-MET/RREB1 axis as a critical oncogenic regulator and a promising therapeutic target in in high-risk MB.
    Journal
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    TGFB1 (Transforming Growth Factor Beta 1) • RREB1 (Ras Responsive Element Binding Protein 1)
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    SU11274
    7ms
    Invasive Melanoma Arising in a BAP1-Inactivated Melanocytic Tumor With NRAS Mutation: A Report of Exceptional Case With Emphasis on Its Genomic Features and Review of the Literature. (PubMed, J Cutan Pathol)
    The patient completed pembrolizumab adjuvant therapy with no evidence of metastasis. This is a rare presentation of BIMT with BAP1 and NRAS mutations, absence of BRAF V600 mutation, and loss of BAP1 immunoreactivity in all lesional cells. Our case adds to the understanding of the histomorphologic and mutational spectrum in BAP1-inactivated melanocytic tumors.
    Journal • PD(L)-1 Biomarker
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    BRAF (B-raf proto-oncogene) • NRAS (Neuroblastoma RAS viral oncogene homolog) • BAP1 (BRCA1 Associated Protein 1) • RREB1 (Ras Responsive Element Binding Protein 1)
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    BRAF V600E • NRAS mutation
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    Keytruda (pembrolizumab)
    8ms
    An epithelioid hemangioendothelioma with a novel RREB1::TFE3 gene fusion. (PubMed, Virchows Arch)
    Histologically, however, the tumor partly revealed an uncommon morphology: on one hand, solid areas of monomorphic epithelioid tumor cells with abundant eosinophilic cytoplasm, and on the other, regions with considerable and unusual pleomorphism and multinucleation, thus not clearly aligning with the prototypical phenotypes of the classical molecular subtypes. In summary, this case expands the molecular spectrum of EHE and supports further investigation into its molecular heterogeneity.
    Journal
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    YAP1 (Yes associated protein 1) • TFE3 (Transcription Factor Binding To IGHM Enhancer 3) • WWTR1 (WW Domain Containing Transcription Regulator 1) • CAMTA1 (Calmodulin Binding Transcription Activator 1) • RREB1 (Ras Responsive Element Binding Protein 1)
    8ms
    First Report of a Novel Pathogenic Variant in the RREB1 Gene Associated With Obesity and Metabolic Syndrome. (PubMed, Clin Genet)
    Additionally, the patient's neurodevelopmental delay aligns with previously reported findings of RREB1 loss-of-function variants. These results highlight the need for further research to fully understand the metabolic implications of RREB1 gene loss, with this study providing valuable insights for future investigations.
    Journal
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    RREB1 (Ras Responsive Element Binding Protein 1)
    9ms
    Beyond Hybrid Morphology: A Large Series of Fusion-Driven Benign Peripheral Nerve Sheath Tumors Including 5 Tumors with Novel Fusions. (PubMed, Mod Pathol)
    Lastly, one tumor with a novel SRF::MYOCD fusion displayed morphologic features reminiscent of desmoplastic melanoma while exhibiting a combined neural and smooth muscle phenotype. Our data expands on the morphologic and molecular spectrum of fusion-driven hybrid peripheral nerve sheath tumors, including 5 previously undescribed fusions, and further expands on non-CNS schwannomas with VGLL3 fusions.
    Journal
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    KANK1 (KN Motif And Ankyrin Repeat Domains 1) • CDK5RAP2 (CDK5 Regulatory Subunit Associated Protein 2) • NCOA2 (Nuclear Receptor Coactivator 2) • RREB1 (Ras Responsive Element Binding Protein 1) • TEAD1 (TEA Domain Transcription Factor 1) • VGLL3 (Vestigial Like Family Member 3)
    12ms
    An extralingual Ectomesenchymal chondromyxoid tumor with RREB1::MRTFB fusion: a rare case report of plantar fascia involvement. (PubMed, Diagn Pathol)
    We report, for the first time, a mesenchymal chondromyxoid tumor with an RREB1::MRTFB fusion gene occurring in the foot. This case expands the known distribution of ECT beyond the tongue. Accurate differential diagnosis should rely on thorough histological assessment, combined with immunohistochemical and molecular analyses.
    Journal
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    RAS (Rat Sarcoma Virus) • NCAM1 (Neural cell adhesion molecule 1) • SOX10 (SRY-Box 10) • TP63 (Tumor protein 63) • GFAP (Glial Fibrillary Acidic Protein) • RREB1 (Ras Responsive Element Binding Protein 1)
    12ms
    Somatic Genomic Profiling of Pancreatic Ductal Adenocarcinomas From a Diverse Cohort of Patients. (PubMed, Pancreas)
    Genomic analysis of PDAC tumors from B/AA and White patients demonstrate similarities in mutation frequencies. Larger studies are needed to further understand molecular characterizations across continental subpopulations. This study provides further rationale for equitable representation of diverse patients in genomic databases and clinical trials.
    Journal • BRCA Biomarker
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    KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • TP53 (Tumor protein P53) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • ARID1A (AT-rich interaction domain 1A) • SMAD4 (SMAD family member 4) • KDM6A (Lysine Demethylase 6A) • RREB1 (Ras Responsive Element Binding Protein 1)
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    TP53 mutation • KRAS mutation • BRAF mutation • ATM mutation • ARID1A mutation
    12ms
    The SUMOylated RREB1 interacts with KDM1A to induce 5-fluorouracil resistance via upregulating thymidylate synthase and activating DNA damage response pathway in colorectal cancer. (PubMed, MedComm (2020))
    Moreover, KDM1A knockdown improved the DNA damage and reduced RREB1-mediated resistance to 5-FU. These findings provide new insights into RREB1-mediated chemotherapy responses in CRC and indicate RREB1 is a potential target for overcoming 5-FU resistance.
    Journal
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    KDM1A (Lysine Demethylase 1A) • TYMS (Thymidylate Synthetase) • CHEK1 (Checkpoint kinase 1) • RREB1 (Ras Responsive Element Binding Protein 1)
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    5-fluorouracil
    1year
    Clinical, morphologic and genomic findings in Spitz tumors with RET fusion: a series of 31 cases. (PubMed, Mod Pathol)
    DNA and RNA sequencing detected several further genomic alterations, including POU2F3 overexpression in 3 highly pigmented lesions. Further studies are needed to confirm possible correlations between the microscopic features and a particular fusion partner (or additional genetic events) in RET-fused Spitz neoplasms.
    Journal
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    RET (Ret Proto-Oncogene) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • KIF5B (Kinesin Family Member 5B) • CCND1 (Cyclin D1) • CCDC6 (Coiled-Coil Domain Containing 6) • LMNA (Lamin A/C) • NCOA4 (Nuclear Receptor Coactivator 4) • ERC1 (ELKS/RAB6-Interacting/CAST Family Member 1) • POU2F3 (POU Class 2 Homeobox 3) • RREB1 (Ras Responsive Element Binding Protein 1)
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    RET fusion
    1year
    BRCA1-Associated Protein-1 Inactivated Melanoma Arising in a Pre-existing Nevus With ALK Fusion and Low Tumor Mutational Burden. (PubMed, Am J Dermatopathol)
    The tumor was completely excised with negative margins. The patient is doing well at 17 months follow-up with no signs of recurrence.
    Journal • Tumor mutational burden • BRCA Biomarker
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    BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • TP53 (Tumor protein P53) • TMB (Tumor Mutational Burden) • FGFR3 (Fibroblast growth factor receptor 3) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • TERT (Telomerase Reverse Transcriptase) • BAP1 (BRCA1 Associated Protein 1) • VHL (von Hippel-Lindau tumor suppressor) • RAF1 (Raf-1 Proto-Oncogene Serine/Threonine Kinase) • PRAME (Preferentially Expressed Antigen In Melanoma) • MAP2K7 (Mitogen-Activated Protein Kinase Kinase 7) • RREB1 (Ras Responsive Element Binding Protein 1)
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    TP53 mutation • BRAF V600E • BRAF V600 • ALK positive • TMB-L • ALK fusion • ALK mutation