Tumor necrosis factor receptor superfamily, member 13C (TNFRSF13C), also known as B-cell-activating factor receptor (BAFFR) or CD268 antigen, is a member of the tumor necrosis factor receptor superfamily. BAFFR promotes B cell survival and is crucial for B cell maturation. BAFF binds to three TNF receptor superfamily members: B-cell maturation antigen (BCMA/TNFRSF17), transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI/TNFRSF13B), and BAFF receptor (BAFFR/BR3/TNFRSF13C). These receptors are type III transmembrane proteins without a signal peptide. BAFFR is highly expressed in the spleen, lymph nodes, and resting B cells, and at lower levels in activated B cells, resting CD4+ T cells, the thymus, and peripheral blood leukocytes. BAFF knockout mice lack mature B cells, as do A/WySnJ mice with defects in BAFF-R intracellular signaling, indicating that BAFFR is the critical receptor for BAFF during B lymphopoiesis. It is hypothesized that elevated levels of BAFFR/TNFRSF13C (CD268) may contribute to autoimmune disease pathogenesis by enhancing the survival of autoreactive B cells.
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