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    GENE:

    TNFRSF13B (TNF Receptor Superfamily Member 13B)

    i
    Other names: TNFRSF13B, TNF Receptor Superfamily Member 13B, TACI, CD267, IGAD2, Tumor Necrosis Factor Receptor Superfamily Member 13B, Transmembrane Activator And CAML Interactor, Tumor Necrosis Factor Receptor Superfamily, Member 13B, Tumor Necrosis Factor Receptor 13B, CD267 Antigen, TNFRSF14B, CVID2, CVID, RYZN
    Associations

    News

    Trials
    11d
    Leniolisib for Immune Dysregulation in CVID (clinicaltrials.gov)
    P2, N=20, Active, not recruiting, Pharming Technologies B.V. | Recruiting --> Active, not recruiting
    Enrollment closed
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    PTEN (Phosphatase and tensin homolog) • CD20 (Membrane Spanning 4-Domains A1) • CTNNB1 (Catenin (cadherin-associated protein), beta 1) • IKZF1 (IKAROS Family Zinc Finger 1) • CTLA4 (Cytotoxic T-Lymphocyte Associated Protein 4) • CARD11 (Caspase Recruitment Domain Family Member 11) • NFKB2 (Nuclear Factor Kappa B Subunit 2) • TNFRSF13C (TNF Receptor Superfamily Member 13C) • CD81 (CD81 Molecule) • SEC61A1 (SEC61 Translocon Subunit Alpha 1) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    26d
    GSTA1 deficiency drives neuroendocrine differentiation via TNFRSF13B/c-FOS/CHGA axis in prostate cancer. (PubMed, Int J Biol Sci)
    Following enzalutamide (ENZ) treatment, GSTA1 expression is inhibited...TNFRSF13B induces c-Fos expression, forming a transcriptional complex with c-Jun, thereby regulating chromogranin A (CHGA) and promoting the neuroendocrine phenotype. Our study suggested that GSTA1 deficiency leads to elevated ROS levels and activation of TNFRSF13B and c-FOS, which subsequently transcriptionally regulate CHGA and ultimately drive neuroendocrine differentiation in PCa.
    Journal
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    TNFA (Tumor Necrosis Factor-Alpha) • CHGA (Chromogranin A) • FOS (Fos Proto-Oncogene AP-1 Transcription Factor Subunit 2) • JUN (Jun proto-oncogene) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
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    Xtandi (enzalutamide)
    26d
    Identification of CD320, SLC44A1 and TNFRSF13B as potential novel therapeutic targets for CAR T-cell therapy in multiple myeloma. (PubMed, Front Med (Lausanne))
    CD320, SLC44A1, and TNFRSF13B are promising, clinically relevant targets for CAR T-cell therapy in MM. Their stage-specific expression and prognostic significance support their potential to enhance existing immunotherapeutic strategies.
    Journal • IO biomarker
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    CD38 (CD38 Molecule) • SDC1 (Syndecan 1) • FCGR2B (Fc Fragment Of IgG Receptor IIb) • CD59 (CD59 Molecule) • SLAMF7 (SLAM Family Member 7) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    2ms
    TNFRSF13B Variant-Induced TACI Dysregulation Underlies CAEBV Pathogenesis. (PubMed, J Clin Immunol)
    Gene silencing experiments indicated that XBP-1 might be involved in the TACI-mediated regulation of EBV lytic activities in EBV-immortalized B cells. This study underscores the impact of TNFRSF13B variants on EBV infection and host immune responses, offering insights into CAEBV pathogenesis and potential therapeutic strategies.
    Journal
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    TNFA (Tumor Necrosis Factor-Alpha) • IL10 (Interleukin 10) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    5ms
    Single-cell profiling of ERBB family receptors identifies ERBB3 as a key regulator in head and neck squamous cell carcinoma progression. (PubMed, Discov Oncol)
    Flow cytometry analysis in a 4NQO-induced mouse model showed that ERBB3 inhibition reduced CCDC50⁺ B cells while restoring MHC-II expression, indicating a shift toward immune activation. These findings highlight a dual role of ERBB3 in HNSCC, acting both as an oncogenic contributor and an immune-modulatory regulator, and position ERBB3 as a promising context-dependent therapeutic target.
    Journal
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    ERBB3 (V-erb-b2 avian erythroblastic leukemia viral oncogene homolog 3) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    5ms
    Malignancy in Adults with Inborn Errors of Immunity: A Retrospective Single-Center Study. (PubMed, J Clin Immunol)
    Findings confirm that NHL, particularly DLBCL, is the most prevalent malignancy in this cohort. Given the link between malignancies and underlying IEI, immunologic evaluation is recommended, particularly for NHL patients. The observed predominance of hematologic malignancies among CVID patients and the association with lymphoproliferation further emphasize the need for heightened malignancy surveillance and early immunologic workup in this subgroup. Further research on biomarkers for malignancy prediction in IEI is warranted.
    Retrospective data • Journal
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    NF1 (Neurofibromin 1) • CHEK2 (Checkpoint kinase 2) • CARD11 (Caspase Recruitment Domain Family Member 11) • PLCG2 (Phospholipase C Gamma 2) • SPI1 (Spi-1 Proto-Oncogene) • NFKBIA (NFKB Inhibitor Alpha 2) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    5ms
    Re-evaluation of the contribution of TNFRSF13B variants to antibody deficiency. (PubMed, J Hum Immun)
    We identified (i) biallelic mutations in TNFRSF13B, (ii) the HLA-class II marker (DPA1*03), and (iii) multiple single nucleotide polymorphisms in known B-cell related genes, as additional genetic risk factors. Moreover, pathogenic mutations in other known IEI genes were presented in 16% of patients with heterozygous TNFRSF13B variants.
    Journal
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    TNFA (Tumor Necrosis Factor-Alpha) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    6ms
    Cutaneous AL-Type Amyloidomas as a Variant of Primary Cutaneous Marginal Zone Lymphoma: A Series of 30 Patients. (PubMed, Am J Dermatopathol)
    These lesions exhibit distinct histopathologic features, such as amyloid deposition, and have a favorable prognosis with minimal risk of progression to systemic amyloidosis. This study supports the hypothesis that AL-amyloidomas are part of the broader spectrum of indolent B-cell lymphomas.
    Journal
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    TNFRSF13B (TNF Receptor Superfamily Member 13B)
    8ms
    Exploring germline variants in genes associated with inborn errors of immunity and inherited bone marrow failure syndromes in pediatric hematological malignancies. (PubMed, Eur J Cancer)
    This study assessed for the first time in an unbiased and comprehensive manner the role of IEI/IBMFS in childhood hematological malignancy predisposition. Although the overall yield of this exploratory study was limited, our findings support the importance of research on childhood cancer predisposition at the intersection of hematological malignancies and IEI/IBMFS. We identified several variants in both dominant and recessive genes of which it would be interesting to investigate their causality. However, for now, sequencing of IEI/IBMFS-associated genes should be restricted to research context or in case of clinical suspicion.
    Journal
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    TNFRSF13B (TNF Receptor Superfamily Member 13B)
    9ms
    Familial aspects of multiple myeloma and Waldenström macroglobulinemia: understanding the predisposition in relatives and the importance of early diagnosis. (PubMed, Leuk Lymphoma)
    Polygenic inheritance also plays a significant role, with risk loci influencing plasma cell survival and transformation. These findings have direct implications for risk assessment, early diagnosis, and surveillance in at-risk relatives.
    Review • Journal • BRCA Biomarker
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    TP53 (Tumor protein P53) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • POT1 (Protection of telomeres 1) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    12ms
    Pathogenic germline variants in cancer predisposition genes in patients with multiple primary cancers in an Asian population and the role of extended panel genetic testing. (PubMed, ESMO Open)
    Patients with MPC were more likely to harbour a PGV. Extended testing improved PGV detection rates, particularly for less well-known cancer predisposition genes.
    Journal • BRCA Biomarker
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    TP53 (Tumor protein P53) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • ATM (ATM serine/threonine kinase) • HRD (Homologous Recombination Deficiency) • PALB2 (Partner and localizer of BRCA2) • TET2 (Tet Methylcytosine Dioxygenase 2) • MLH1 (MutL homolog 1) • MSH6 (MutS homolog 6) • MSH2 (MutS Homolog 2) • PMS2 (PMS1 protein homolog 2) • CHEK2 (Checkpoint kinase 2) • BRIP1 (BRCA1 Interacting Protein C-terminal Helicase 1) • RAD51C (RAD51 paralog C) • RAD51D (RAD51 paralog D) • MBD4 (Methyl-CpG Binding Domain 4, DNA Glycosylase) • BARD1 (BRCA1 Associated RING Domain 1) • DDX41 (DEAD-Box Helicase 41) • FANCL (FA Complementation Group L) • CTNNA1 (Catenin Alpha 1) • RECQL4( RecQ Like Helicase 4) • SPINK1 (Serine peptidase inhibitor, kazal type 1) • CDKN1C (Cyclin Dependent Kinase Inhibitor 1C) • TNFRSF13B (TNF Receptor Superfamily Member 13B)
    1year
    Cancer-secreted exosomal miR-1246 promotes colorectal cancer liver metastasis by activating hepatic stellate cells. (PubMed, Mol Med)
    Our study reveals the role of CRC-secreted exosomal miR-1246 in triggering HSC activation and reprogramming the TME, ultimately facilitating liver metastasis in CRC patients. Exosomal miR-1246 could serve as a potential non-invasive biomarker for predicting colorectal cancer liver metastasis, enhancing our understanding of CRC-associated liver metastases.
    Journal
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    TGFB1 (Transforming Growth Factor Beta 1) • TLR4 (Toll Like Receptor 4) • MIR1246 (MicroRNA 1246) • TNFRSF13B (TNF Receptor Superfamily Member 13B) • TNFSF13 (TNF Superfamily Member 13)