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Gene Review

TNFRSF13C  -  tumor necrosis factor receptor superfamily...

Homo sapiens

Synonyms: B-cell-activating factor receptor, BAFF receptor, BAFF-R, BAFFR, BLyS receptor 3, ...
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Disease relevance of TNFRSF13C


High impact information on TNFRSF13C

  • The chromosomes, especially the Balbiani rings (BR2, BR1 and BR3), were examined during periods of stimulated and repressed RNA synthesis [6].
  • Here we determine the crystal structures of sTALL-1 complexed with the extracellular domains of BCMA and BAFF-R at 2.6 and 2.5 A, respectively [7].
  • Thus, BAFF-R appears to be the principal receptor for BAFF-mediated mature B cell survival [8].
  • Consistent with this, the BAFF-R locus is disrupted in A/WySnJ mice, which display a B cell phenotype qualitatively similar to that of the BAFF-deficient mice [8].
  • BAFF was localized in astrocytes close to BAFF-R-expressing immune cells [9].

Chemical compound and disease context of TNFRSF13C

  • An analytical strategy combining fractal geometry and grey-level co-occurrence matrix (GLCM) statistics was devised to investigate ultrastructural changes in oestrogen-insensitive SK-BR3 human breast cancer cells undergoing apoptosis in vitro [10].

Biological context of TNFRSF13C


Anatomical context of TNFRSF13C


Associations of TNFRSF13C with chemical compounds

  • However, the extracellular domain (ECD) of BR3 consists of only a partial CRD, with cysteine spacing distinct from other modules described previously [2].
  • Alanine scanning and crystallographic structural analysis of the CB3s/BR3 complex reveal that CB3s mimics BAFF by interacting with a similar region of the BR3 surface [19].
  • Reaction of vanadocene [V(Cp)2] with "activated" nitrile R1CN.L (L: Lewis acid), obtained by the reaction of borane adducts (L = BR3; R = C6F5, 2,6-F2C6H3, 3,4,5-F3C6H2) with nitriles (CH3CN, F3CC6H4CN), yields the borane adduct of vanada(IV)azirine complexes [V(Cp)2(eta 2-R1C = N.L)] [20].
  • SK-BR3 cells entered the early stage of apoptosis within 24 h of treatment with calcimycin, which induced detectable changes in nuclear components, as documented by increased values of most GLCM parameters and by the general reduction of the fractal dimensions [10].
  • Mice injected with MDA-231 BR3 into the carotid artery were treated with the VEGF-receptor tyrosine kinase inhibitor PTK787/Z 222584 [21].

Physical interactions of TNFRSF13C

  • In this study, we report the crystal structure of a 24-residue fragment of the cytoplasmic portion of BAFF-R bound in complex with TRAF3 [15].
  • However, BAFF-R binds only one TRAF adaptor, TRAF3, and this interaction negatively regulates activation of NF-kappaB [15].
  • BAFF and APRIL bind to BAFF receptor and TACI and are major B-cell survival factors [22].

Regulatory relationships of TNFRSF13C

  • Moreover, overexpression of TRAF3 inhibits BAFF-R-mediated NF-kappaB activation and IL-10 production [3].

Other interactions of TNFRSF13C

  • Key molecular contacts promote recognition of the BAFF receptor by TNF receptor-associated factor 3: implications for intracellular signaling regulation [15].
  • The recognition motif (162)PVPAT(166) in BAFF-R is accommodated in the same binding crevice on TRAF3 that binds two related TNFRs, CD40 and LTbetaR, but is presented in a completely different structural framework [15].
  • In the C-terminal arm, key stabilizing contacts are made, including critical hydrogen bonds with Gln(379) in TRAF3 that define the molecular basis for selective binding of BAFF-R solely to this member of the TRAF family [15].
  • The BR3 fold is analogous to the first half of a canonical TNFR CRD but is stabilized by an additional noncanonical disulfide bond [2].
  • Expression and occupancy of BAFF-R on B cells in systemic lupus erythematosus [23].

Analytical, diagnostic and therapeutic context of TNFRSF13C


  1. Expression of BCMA, TACI, and BAFF-R in multiple myeloma: a mechanism for growth and survival. Novak, A.J., Darce, J.R., Arendt, B.K., Harder, B., Henderson, K., Kindsvogel, W., Gross, J.A., Greipp, P.R., Jelinek, D.F. Blood (2004) [Pubmed]
  2. BAFF/BLyS receptor 3 comprises a minimal TNF receptor-like module that encodes a highly focused ligand-binding site. Gordon, N.C., Pan, B., Hymowitz, S.G., Yin, J., Kelley, R.F., Cochran, A.G., Yan, M., Dixit, V.M., Fairbrother, W.J., Starovasnik, M.A. Biochemistry (2003) [Pubmed]
  3. TNFR-associated factor-3 is associated with BAFF-R and negatively regulates BAFF-R-mediated NF-kappa B activation and IL-10 production. Xu, L.G., Shu, H.B. J. Immunol. (2002) [Pubmed]
  4. Mutational analysis of human BAFF receptor TNFRSF13C (BAFF-R) in patients with common variable immunodeficiency. Losi, C.G., Silini, A., Fiorini, C., Soresina, A., Meini, A., Ferrari, S., Notarangelo, L.D., Lougaris, V., Plebani, A. J. Clin. Immunol. (2005) [Pubmed]
  5. BAFF-R, the major B cell-activating factor receptor, is expressed on most mature B cells and B-cell lymphoproliferative disorders. Rodig, S.J., Shahsafaei, A., Li, B., Mackay, C.R., Dorfman, D.M. Hum. Pathol. (2005) [Pubmed]
  6. RNA polymerase B in polytene chromosomes: immunofluorescent and autoradiographic analysis during stimulated and repressed RNA synthesis. Sass, H. Cell (1982) [Pubmed]
  7. Ligand-receptor binding revealed by the TNF family member TALL-1. Liu, Y., Hong, X., Kappler, J., Jiang, L., Zhang, R., Xu, L., Pan, C.H., Martin, W.E., Murphy, R.C., Shu, H.B., Dai, S., Zhang, G. Nature (2003) [Pubmed]
  8. BAFF-R, a newly identified TNF receptor that specifically interacts with BAFF. Thompson, J.S., Bixler, S.A., Qian, F., Vora, K., Scott, M.L., Cachero, T.G., Hession, C., Schneider, P., Sizing, I.D., Mullen, C., Strauch, K., Zafari, M., Benjamin, C.D., Tschopp, J., Browning, J.L., Ambrose, C. Science (2001) [Pubmed]
  9. BAFF is produced by astrocytes and up-regulated in multiple sclerosis lesions and primary central nervous system lymphoma. Krumbholz, M., Theil, D., Derfuss, T., Rosenwald, A., Schrader, F., Monoranu, C.M., Kalled, S.L., Hess, D.M., Serafini, B., Aloisi, F., Wekerle, H., Hohlfeld, R., Meinl, E. J. Exp. Med. (2005) [Pubmed]
  10. Nuclear patterns of human breast cancer cells during apoptosis: characterisation by fractal dimension and co-occurrence matrix statistics. Losa, G.A., Castelli, C. Cell Tissue Res. (2005) [Pubmed]
  11. BAFF supports human B cell differentiation in the lymphoid follicles through distinct receptors. Zhang, X., Park, C.S., Yoon, S.O., Li, L., Hsu, Y.M., Ambrose, C., Choi, Y.S. Int. Immunol. (2005) [Pubmed]
  12. BAFF and APRIL support chronic lymphocytic leukemia B-cell survival through activation of the canonical NF-{kappa}B pathway. Endo, T., Nishio, M., Enzler, T., Cottam, H.B., Fukuda, T., James, D.F., Karin, M., Kipps, T.J. Blood (2007) [Pubmed]
  13. Impact of the BAFF/BR3 axis on B cell survival, germinal center maintenance and antibody production. Kalled, S.L. Semin. Immunol. (2006) [Pubmed]
  14. The differential expression of LCK and BAFF-receptor and their role in apoptosis in human lymphomas. Paterson, J.C., Tedoldi, S., Craxton, A., Jones, M., Hansmann, M.L., Collins, G., Roberton, H., Natkunam, Y., Pileri, S., Campo, E., Clark, E.A., Mason, D.Y., Marafioti, T. Haematologica (2006) [Pubmed]
  15. Key molecular contacts promote recognition of the BAFF receptor by TNF receptor-associated factor 3: implications for intracellular signaling regulation. Ni, C.Z., Oganesyan, G., Welsh, K., Zhu, X., Reed, J.C., Satterthwait, A.C., Cheng, G., Ely, K.R. J. Immunol. (2004) [Pubmed]
  16. B cell-activating factor belonging to the TNF family (BAFF)-R is the principal BAFF receptor facilitating BAFF costimulation of circulating T and B cells. Ng, L.G., Sutherland, A.P., Newton, R., Qian, F., Cachero, T.G., Scott, M.L., Thompson, J.S., Wheway, J., Chtanova, T., Groom, J., Sutton, I.J., Xin, C., Tangye, S.G., Kalled, S.L., Mackay, F., Mackay, C.R. J. Immunol. (2004) [Pubmed]
  17. The thymus is a source of B-cell-survival factors-APRIL and BAFF-in myasthenia gravis. Thangarajh, M., Masterman, T., Helgeland, L., Rot, U., Jonsson, M.V., Eide, G.E., Pirskanen, R., Hillert, J., Jonsson, R. J. Neuroimmunol. (2006) [Pubmed]
  18. The role of BLyS/BLyS receptors in anti-chromatin B cell regulation. Hondowicz, B.D., Alexander, S.T., Quinn, W.J., Pagán, A.J., Metzgar, M.H., Cancro, M.P., Erikson, J. Int. Immunol. (2007) [Pubmed]
  19. Synthetic anti-BR3 antibodies that mimic BAFF binding and target both human and murine B cells. Lee, C.V., Hymowitz, S.G., Wallweber, H.J., Gordon, N.C., Billeci, K.L., Tsai, S.P., Compaan, D.M., Yin, J., Gong, Q., Kelley, R.F., Deforge, L.E., Martin, F., Starovasnik, M.A., Fuh, G. Blood (2006) [Pubmed]
  20. Reactivity of vanadocene with a nitrile -C identical to N bond activated by a tris(fluorophenyl)borane as Lewis acid: formation of borane adducts of vanada(IV)azirine complexes--EPR evidence for an intramolecular C-F...V interaction. Choukroun, R., Lorber, C., Donnadieu, B. Chemistry (Weinheim an der Bergstrasse, Germany) (2002) [Pubmed]
  21. Vascular endothelial growth factor expression promotes the growth of breast cancer brain metastases in nude mice. Kim, L.S., Huang, S., Lu, W., Lev, D.C., Price, J.E. Clin. Exp. Metastasis (2004) [Pubmed]
  22. Survival and proliferation factors of normal and malignant plasma cells. Klein, B., Tarte, K., Jourdan, M., Mathouk, K., Moreaux, J., Jourdan, E., Legouffe, E., De Vos, J., Rossi, J.F. Int. J. Hematol. (2003) [Pubmed]
  23. Expression and occupancy of BAFF-R on B cells in systemic lupus erythematosus. Carter, R.H., Zhao, H., Liu, X., Pelletier, M., Chatham, W., Kimberly, R., Zhou, T. Arthritis Rheum. (2005) [Pubmed]
  24. Elevated serum BAFF levels in patients with systemic sclerosis: enhanced BAFF signaling in systemic sclerosis B lymphocytes. Matsushita, T., Hasegawa, M., Yanaba, K., Kodera, M., Takehara, K., Sato, S. Arthritis Rheum. (2006) [Pubmed]
  25. Cutting edge: B cell receptor signals regulate BLyS receptor levels in mature B cells and their immediate progenitors. Smith, S.H., Cancro, M.P. J. Immunol. (2003) [Pubmed]
  26. BAFF binding to T cell-expressed BAFF-R costimulates T cell proliferation and alloresponses. Ye, Q., Wang, L., Wells, A.D., Tao, R., Han, R., Davidson, A., Scott, M.L., Hancock, W.W. Eur. J. Immunol. (2004) [Pubmed]
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