The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

RBPJ  -  recombination signal binding protein for...

Homo sapiens

Synonyms: AOS3, CBF-1, CBF1, IGKJRB, IGKJRB1, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of RBPJ

  • Epstein-Barr virus immortalization: Notch2 interacts with CBF1 and blocks differentiation [1].
  • The regulation of K14/vGPCR transcripts by RBP-J raises the possibility that other modulators of Notch signaling might be able to induce expression of this RNA outside the context of lytic KSHV replication [2].
  • Neither EBNA2-TAT, nor a mutant peptide with a 2-aa substitution that was unable to block the EBNA2-CBF1 interaction, significantly affected the growth of non-EBNA2-expressing EBV(-) B cells or Burkitt's lymphoma Akata cells [3].
  • OBJECTIVES: To understand the functional consequences of a recurrent CADASIL mutation on furin processing, cell surface expression, ligand binding, and activation of a downstream effector CBF1 by the Notch3 receptor [4].
  • Two immunogenic proteins of 27 (CBF1) and 29 (CBF2) kDa from enteropathogenic Campylobacter species appear to bind to mammalian cells [5].

High impact information on RBPJ

  • EBNA2 is targeted to responsive promoters through interaction with a cellular DNA binding protein, C promoter binding factor 1 (CBF1) [6].
  • CBF1 is identical to a protein thought to be involved in immunoglobulin gene rearrangement, RBPJ kappa [7].
  • Moreover, we show that coexpression with MAM and CBF1 strongly enhances phosphorylation and proteolytic turnover of the Notch ICD in vivo [8].
  • Activation of the Notch-regulated transcription factor CBF1/RBP-Jkappa through the 13SE1A oncoprotein [9].
  • Similarly, the Epstein Barr viral oncoprotein EBNA2, which is required for B-cell immortalization, activates genes through CBF1 [9].

Chemical compound and disease context of RBPJ


Biological context of RBPJ


Anatomical context of RBPJ

  • Notch-mediated CBF-1/RBP-J{kappa}-dependent regulation of human vascular smooth muscle cell phenotype in vitro [13].
  • We speculate that the Notch4/Int3 ICD-induced block to mammary gland development and tumorigenesis are consequences of an increasing gradient of CBF1-dependent Notch4/Int3 signaling [14].
  • Here, we have tested this hypothesis by examining KSHV infection of RBP-J kappa-null murine fibroblasts [15].
  • In this paper,we show that CBF1 from cell lines of different origin is able to bind to the[kappa]B site of the IL-6 promoter [16].
  • When epithelial stem cells were plated on the bound ligand, the Notch/CBF-1 signaling pathway was stimulated and the cells upregulated both intermediate- and late-stage differentiation markers [17].

Associations of RBPJ with chemical compounds

  • Notch1 signaling, assessed by measuring the activity of CBF1, a downstream transcription factor, was impaired but not abolished by the PS1 aspartate mutations or gamma-secretase inhibitors [18].
  • N1(deltaEC) dramatically increased chloramphenicol acetyltransferase activity in these cells, indicating functional coupling of Notch1 and CBF1 [19].
  • The EBNA2-TAT peptide blocked EBNA2-CBF1 interaction in an in vitro GST affinity assay and labeling with fluorescein confirmed that the EBNA2-TAT peptide efficiently entered cultured B cells [3].
  • By electrophoretic mobility shift assays, we identified a transcription factor complex (C1) that binds sequence specific to one known and 4 newly identified putative CBF1 recognition sites in the CD23a core promoter region [10].
  • We further show that ABA induces the expression of Cat1 via the interaction between ABRE2 and one of its binding proteins, CBF1 (Cat1 binding factor 1) [20].

Physical interactions of RBPJ


Regulatory relationships of RBPJ

  • These findings suggest that Notch promotes changes in hVSMC phenotype via activation of CBF-1/RBP-Jkappa-dependent pathways in vitro and contributes to the phenotypic response of VSMCs to cyclic strain-induced changes in VSMC differentiation [13].
  • The transcription factor YY1 indirectly regulated the transcriptional activity of the wild-type CBF1-response elements via the direct interaction of N1IC and CBF1 [23].
  • Moreover, we found that overexpression of RING1 together with KyoT2 in cells inhibited transactivation of RBP-J by NIC [24].

Other interactions of RBPJ

  • CIR, a corepressor linking the DNA binding factor CBF1 to the histone deacetylase complex [11].
  • The activated intracellular form of Notch, NotchIC, translocates to the nucleus, where it targets the DNA binding protein CBF1 [12].
  • In competition binding experiments SMRT displaced NotchIC from CBF1 and from SKIP [12].
  • These results support a model in which the binding of NIC-1 to CBF1 is required for AP-1 repression and reveal a powerful cell-sensing mechanism that suppresses the levels of transcriptionally competent NIC-1 [25].
  • c-Myc substitutes for Notch1-CBF1 functions in cooperative transformation with papillomavirus oncogenes [26].

Analytical, diagnostic and therapeutic context of RBPJ


  1. Epstein-Barr virus immortalization: Notch2 interacts with CBF1 and blocks differentiation. Hsieh, J.J., Nofziger, D.E., Weinmaster, G., Hayward, S.D. J. Virol. (1997) [Pubmed]
  2. RBP-J (CSL) is essential for activation of the K14/vGPCR promoter of Kaposi's sarcoma-associated herpesvirus by the lytic switch protein RTA. Liang, Y., Ganem, D. J. Virol. (2004) [Pubmed]
  3. Inhibition of Epstein-Barr virus-induced growth proliferation by a nuclear antigen EBNA2-TAT peptide. Farrell, C.J., Lee, J.M., Shin, E.C., Cebrat, M., Cole, P.A., Hayward, S.D. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  4. Functional analysis of a recurrent missense mutation in Notch3 in CADASIL. Haritunians, T., Chow, T., De Lange, R.P., Nichols, J.T., Ghavimi, D., Dorrani, N., St Clair, D.M., Weinmaster, G., Schanen, C. J. Neurol. Neurosurg. Psychiatr. (2005) [Pubmed]
  5. Isolation and characterization of two Campylobacter glycine-extracted proteins that bind to HeLa cell membranes. Kervella, M., Pagès, J.M., Pei, Z., Grollier, G., Blaser, M.J., Fauchère, J.L. Infect. Immun. (1993) [Pubmed]
  6. Masking of the CBF1/RBPJ kappa transcriptional repression domain by Epstein-Barr virus EBNA2. Hsieh, J.J., Hayward, S.D. Science (1995) [Pubmed]
  7. Mediation of Epstein-Barr virus EBNA2 transactivation by recombination signal-binding protein J kappa. Henkel, T., Ling, P.D., Hayward, S.D., Peterson, M.G. Science (1994) [Pubmed]
  8. Mastermind mediates chromatin-specific transcription and turnover of the Notch enhancer complex. Fryer, C.J., Lamar, E., Turbachova, I., Kintner, C., Jones, K.A. Genes Dev. (2002) [Pubmed]
  9. Activation of the Notch-regulated transcription factor CBF1/RBP-Jkappa through the 13SE1A oncoprotein. Ansieau, S., Strobl, L.J., Leutz, A. Genes Dev. (2001) [Pubmed]
  10. Notch2 is involved in the overexpression of CD23 in B-cell chronic lymphocytic leukemia. Hubmann, R., Schwarzmeier, J.D., Shehata, M., Hilgarth, M., Duechler, M., Dettke, M., Berger, R. Blood (2002) [Pubmed]
  11. CIR, a corepressor linking the DNA binding factor CBF1 to the histone deacetylase complex. Hsieh, J.J., Zhou, S., Chen, L., Young, D.B., Hayward, S.D. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  12. SKIP, a CBF1-associated protein, interacts with the ankyrin repeat domain of NotchIC To facilitate NotchIC function. Zhou, S., Fujimuro, M., Hsieh, J.J., Chen, L., Miyamoto, A., Weinmaster, G., Hayward, S.D. Mol. Cell. Biol. (2000) [Pubmed]
  13. Notch-mediated CBF-1/RBP-J{kappa}-dependent regulation of human vascular smooth muscle cell phenotype in vitro. Morrow, D., Scheller, A., Birney, Y.A., Sweeney, C., Guha, S., Cummins, P.M., Murphy, R., Walls, D., Redmond, E.M., Cahill, P.A. Am. J. Physiol., Cell Physiol. (2005) [Pubmed]
  14. Mammary development and tumorigenesis in mice expressing a truncated human Notch4/Int3 intracellular domain (h-Int3sh). Raafat, A., Bargo, S., Anver, M.R., Callahan, R. Oncogene (2004) [Pubmed]
  15. Lytic but not latent infection by Kaposi's sarcoma-associated herpesvirus requires host CSL protein, the mediator of Notch signaling. Liang, Y., Ganem, D. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  16. Interaction of the nuclear protein CBF1 with the kappaB site of the IL-6 gene promoter. Palmieri, M., Sasso, M.P., Monese, R., Merola, M., Faggioli, L., Tovey, M., Furia, A. Nucleic Acids Res. (1999) [Pubmed]
  17. Mimicking cell-cell interactions at the biomaterial-cell interface for control of stem cell differentiation. Beckstead, B.L., Santosa, D.M., Giachelli, C.M. Journal of biomedical materials research. Part A. (2006) [Pubmed]
  18. Aspartate mutations in presenilin and gamma-secretase inhibitors both impair notch1 proteolysis and nuclear translocation with relative preservation of notch1 signaling. Berezovska, O., Jack, C., McLean, P., Aster, J.C., Hicks, C., Xia, W., Wolfe, M.S., Kimberly, W.T., Weinmaster, G., Selkoe, D.J., Hyman, B.T. J. Neurochem. (2000) [Pubmed]
  19. Constitutively active human Notch1 binds to the transcription factor CBF1 and stimulates transcription through a promoter containing a CBF1-responsive element. Lu, F.M., Lux, S.E. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  20. Cis-elements and trans-factors that regulate expression of the maize Cat1 antioxidant gene in response to ABA and osmotic stress: H2O2 is the likely intermediary signaling molecule for the response. Guan, L.M., Zhao, J., Scandalios, J.G. Plant J. (2000) [Pubmed]
  21. Functional interaction between the mouse notch1 intracellular region and histone acetyltransferases PCAF and GCN5. Kurooka, H., Honjo, T. J. Biol. Chem. (2000) [Pubmed]
  22. CBF-1 (RBP-Jkappa) binds to the PTEN promoter and regulates PTEN gene expression. Whelan, J.T., Forbes, S.L., Bertrand, F.E. Cell Cycle (2007) [Pubmed]
  23. Association of transcription factor YY1 with the high molecular weight Notch complex suppresses the transactivation activity of Notch. Yeh, T.S., Lin, Y.M., Hsieh, R.H., Tseng, M.J. J. Biol. Chem. (2003) [Pubmed]
  24. RING1 inhibits transactivation of RBP-J by Notch through interaction with LIM protein KyoT2. Qin, H., Wang, J., Liang, Y., Taniguchi, Y., Tanigaki, K., Han, H. Nucleic Acids Res. (2004) [Pubmed]
  25. Evidence that C promoter-binding factor 1 binding is required for Notch-1-mediated repression of activator protein-1. Chu, J., Bresnick, E.H. J. Biol. Chem. (2004) [Pubmed]
  26. c-Myc substitutes for Notch1-CBF1 functions in cooperative transformation with papillomavirus oncogenes. Subramanyam, D., Krishna, S. Virology (2006) [Pubmed]
  27. Assignment of the human gene for KBF2/RBP-Jk to chromosome 9p12-13 and 9q13 by fluorescence in situ hybridization. Tang, X., Saito-Ohara, F., Song, J., Koga, C., Ugai, H., Murakami, H., Ikeuchi, T., Yokoyama, K.K. Jpn. J. Hum. Genet. (1997) [Pubmed]
  28. Physical interaction between a novel domain of the receptor Notch and the transcription factor RBP-J kappa/Su(H). Tamura, K., Taniguchi, Y., Minoguchi, S., Sakai, T., Tun, T., Furukawa, T., Honjo, T. Curr. Biol. (1995) [Pubmed]
  29. Epstein-Barr virus nuclear antigen 2 induces FcRH5 expression through CBF1. Mohan, J., Dement-Brown, J., Maier, S., Ise, T., Kempkes, B., Tolnay, M. Blood (2006) [Pubmed]
WikiGenes - Universities