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FLASH GENE
Symbol TNFRSF13B contributors: mct/npt - updated : 20-09-2013
HGNC name tumor necrosis factor receptor superfamily, member 13B
HGNC id 18153
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • an extracellular domain with a cysteine rich TNFR repeat
  • a transmembrane segment (TM1)
  • intracellular domain interacting with TNFR-associated factor (TRAF)2, 5, and 6
  • a cytoplasmic C terminus
    • HOMOLOGY
    interspecies homolog to murine TacI
    Homologene
    FAMILY
  • lymphocyte-specific member of the tumor necrosis factor (TNF) receptor superfamily
    • CATEGORY signaling
    SUBCELLULAR LOCALIZATION     plasma membrane
    basic FUNCTION
  • playing a crucial role in humoral immunity by interacting with a TNF ligand
  • key regulator of B cell function (Hymowitz 2005)
  • its activation in a B cell line results in nuclear factor kappaB and c-Jun NH(2)-terminal kinase activation (Xia (2000)
  • TRAF-interacting receptor for TNFSF13B, a tumor necrosis factor family member involved in B cell regulation (Xia 2000)
  • triggers immunoglobulin class switching by activating B cells through the adaptor MYD88
  • dual roles for TNFRSF13B in B-cell terminal differentiation
  • has a dual role in B-cell terminal differentiation in limiting the expansion of GC B cells and mediating the survival of plasma cells
  • is likely required for optimal T-cell–dependent humoral immune response
  • is required for the survival of plasma cells and not for their differentiation
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS immunity/defense
    text humoral immune response
    PATHWAY
    metabolism
    signaling signal transduction
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • cyclophilin ligand protein CAML
  • activator of NFAT
  • TNFSF13, TNFSF13B (ligands of TNFRSF13)
  • promotes sustained PRDM1 expression by B cells responding to antigen, which in turn limits B-cell clonal expansion and facilitates differentiation of long-lived antibody-secreting cells
  • TNFRSF13B binds the cytokines TNFSF13B and ANP32B
  • negatively regulates ICOSLG expression on B cells
  • TNFRSF13B plays an important role in plasma cells and signals PRDM1 expression
  • loss of TNFRSF13B leads to increased ICOSLG expression and expands T follicular helper (Tfh) and germinal center (GC) B cells
  • requirement of an intact MYD88-binding site in TNFRSF13B to trigger class switch recombination (CSR)
  • TNFRSF13B is released by ADAM10 and reflects B cell activation in autoimmunity
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) CVID2
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target for B cell-mediated autoimmune diseases such as systemic lupus erythematosus (Xia 2000)
    ANIMAL & CELL MODELS
  • inactivation in mice results in a SLE-like phenotype (Salzer 2007)
  • Taci-/- mice had increased numbers of B cells but exhibited decreased antibody responses to T-cell–independent type II antigens