BAFF receptor (B-cell activating factor receptor, BAFF-R), also known as tumor necrosis factor receptor superfamily member 13C (TNFRSF13C), is a membrane protein of the TNF receptor superfamily which recognizes BAFF. In humans it is encoded by the TNFRSF13Cgene.
B-cell activating factor (BAFF) enhances B-cell survival in vitro and is a regulator of the peripheral B-cell population. The protein encoded by this gene is a receptor for BAFF and is a type III transmembrane protein containing a single extracellular phenylalanine-rich domain. It is thought that this receptor is the principal receptor required for BAFF-mediated mature B-cell survival.
Overexpression of BAFF in mice results in mature B-cell hyperplasia and symptoms of systemic lupus erythematosus (SLE). Also, some SLE patients have increased levels of BAFF in serum. Therefore, it has been proposed that abnormally high levels of BAFF may contribute to the pathogenesis of autoimmune diseases by enhancing the survival of autoreactive B cells.
Treml LS, Crowley JE, Cancro MP (Oct 2006). "BLyS receptor signatures resolve homeostatically independent compartments among naïve and antigen-experienced B cells". Seminars in Immunology18 (5): 297–304. doi:10.1016/j.smim.2006.07.001. PMID16919470.
Kalled SL (Oct 2006). "Impact of the BAFF/BR3 axis on B cell survival, germinal center maintenance and antibody production". Seminars in Immunology18 (5): 290–6. doi:10.1016/j.smim.2006.06.002. PMID16931038.
Yan M, Brady JR, Chan B, Lee WP, Hsu B, Harless S, Cancro M, Grewal IS, Dixit VM (Oct 2001). "Identification of a novel receptor for B lymphocyte stimulator that is mutated in a mouse strain with severe B cell deficiency". Current Biology11 (19): 1547–52. doi:10.1016/S0960-9822(01)00481-X. PMID11591325.
Xu LG, Shu HB (Dec 2002). "TNFR-associated factor-3 is associated with BAFF-R and negatively regulates BAFF-R-mediated NF-kappa B activation and IL-10 production". Journal of Immunology169 (12): 6883–9. doi:10.4049/jimmunol.169.12.6883. PMID12471121.
Kim HM, Yu KS, Lee ME, Shin DR, Kim YS, Paik SG, Yoo OJ, Lee H, Lee JO (May 2003). "Crystal structure of the BAFF-BAFF-R complex and its implications for receptor activation". Nature Structural Biology10 (5): 342–8. doi:10.1038/nsb925. PMID12715002.
Liu Y, Hong X, Kappler J, Jiang L, Zhang R, Xu L, Pan CH, Martin WE, Murphy RC, Shu HB, Dai S, Zhang G (May 2003). "Ligand-receptor binding revealed by the TNF family member TALL-1". Nature423 (6935): 49–56. doi:10.1038/nature01543. PMID12721620.
Gordon NC, Pan B, Hymowitz SG, Yin J, Kelley RF, Cochran AG, Yan M, Dixit VM, Fairbrother WJ, Starovasnik MA (May 2003). "BAFF/BLyS receptor 3 comprises a minimal TNF receptor-like module that encodes a highly focused ligand-binding site". Biochemistry42 (20): 5977–83. doi:10.1021/bi034017g. PMID12755599.