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...

Mus musculus

Synonyms: AI843960, CBF1, Igkjrb, Igkjrb1, Igkrsbp, ...
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

  • Here we report that conditionally disrupting the mouse RBP-J gene in a mosaic pattern to avoid embryonic lethality of RBP-J-deficiency caused hair loss, epidermal hyperkeratinization, and epidermal cyst formation [1].
  • In the course of these studies, it was observed that coinjection of P-CRTs with large numbers of normal CBF1 thymocytes (F1 NTs) significantly reduced the GVHR of the P-CRTs as measured in Simonsen splenomegaly and [3H]TdR splenic proliferation assays [2].
  • In addition, two candidate binding sites for the cellular transcription factor RBP-Jkappa/CBF1 were also identified in one of the Rta-responsive regions, which may play a role in mediating Rta transactivation similar to that observed in some KSHV Rta-responsive genes [3].
  • We have raised monoclonal antibodies against the RBP-J kappa fusion protein which was synthesized in E. coli and unable to bind J kappa-RS [4].
  • At 5 wk after the intraperitoneal infection of murine cytomegalovirus (MCMV) in adult (C3H/He x BALB/c) F1 (CBF1) mice, MCMV-DNA was noted mainly in the salivary glands (SG) [5].

Psychiatry related information on Rbpj

  • We also show that null heterozygous mutations in the downstream cofactor RBP-J result in similarly specific spatial learning and memory deficits [6].

High impact information on Rbpj

  • We also describe vascular defects in embryos homozygous for a mutation in the Rbpsuh gene, which encodes the primary transcriptional mediator of Notch signaling [7].
  • Analysis of the enhancer regulating node-specific Nodal expression revealed the presence of binding sites for the RBP-J protein, the primary transcriptional mediator of Notch signaling [8].
  • Our data demonstrated that Notch/RBP-J signaling regulates gammadelta T cell generation and migration, alphabeta T cell maturation, terminal differentiation of CD4(+) T cells into Th1/Th2 cells, and activation of T cells [9].
  • We found that Msx2-interacting nuclear target protein (MINT) competed with the intracellular region of Notch for binding to a DNA binding protein RBP-J and suppressed the transactivation activity of Notch signaling [10].
  • In addition, in mice with RBP-J-deficient B cells, had no obvious changes in immunoglobulin production in response to Ficoll, lipopolysaccharide or chicken gammaglobulin [11].

Chemical compound and disease context of Rbpj

  • The effect of fexofenadine on systemic anaphylaxis caused by IgE and anti-IgE was also examined in CBF1 mice injected with serum from NC/Nga mice with high IgE levels [12].

Biological context of Rbpj


Anatomical context of Rbpj

  • To address this issue directly, we generated zygotes in which both the maternal and the zygotic expression of Rbpsuh, a key element of the core Notch signaling pathway, were abrogated [17].
  • We showed that inhibition of Notch/RBP-J signaling, by either conditional disruption of the Rbpsuh gene or treatment with a gamma-secretase inhibitor, could give rise to ectopic hair cells in the supporting cell region in organs of Corti from neonatal mouse cochleas where hair cells have not been considered to regenerate after birth [18].
  • The RBP-J kappa gene is highly conserved in a wide variety of species and the Drosophila homologue has been shown to be identical to Suppressor of Hairless [Su(H)] which plays important roles in the development of the peripheral nervous system [14].
  • Inhibition of Notch/RBP-J signaling induces hair cell formation in neonate mouse cochleas [18].
  • The loss of RBP-J at an earlier developmental stage resulted in enhanced generation and accelerated emigration of gammadelta T cells, whereas alphabeta T cell development was arrested at the double-negative 3 stage [9].

Associations of Rbpj with chemical compounds

  • Lymphoid progenitors deficient in RBP-J differentiate into B but not T cells when cultured in 2'-deoxyguanosine-treated fetal thymic lobes by hanging-drop fetal thymus organ culture [19].
  • Class-specific plaque-forming cell (PFC) (gammaM, gamma1, gamma2, and gammaA) responses to type III pneumococcal polysaccharide (SSS-III) were studied in BALB/c x C57BL/6F1 (CBF1) mice with and without induction of an allogeneic effect [20].
  • Metabolism of choline in brain of the aged CBF-1 mouse [21].
  • In order to quantify the changes that occur in the cholinergic central nervous system with aging, we have compared acetylcholine (Ach) formation in brain cortex slice preparations from 2-year-old aged CBF-1 mouse brains and compared the findings with those in 2-4-month-old young adult mouse brain slices [21].
  • Therefore, quantitative morphometry of nerve terminals stained with zinc iodide-osmium was carried out in soleus and extensor digitorum longus (EDL) muscles of CBF-1 mice at various ages from 5 to 32 months [22].

Physical interactions of Rbpj

  • As development proceeds, RBPJL gradually replaces RBPJ in the PTF1 complex bound to Rbpjl and appears on the binding sites for the complex in the promoters of other acinar-specific genes, including those for the secretory digestive enzymes [23].
  • We show that a mutant form of Ptf1a without the ability to bind Rbpj, while retaining its ability to interact with E-protein, is incapable of inducing GABAergic (Pax2)- and suppressing glutamatergic (Tlx3)-expressing cells in the chick and mouse neural tube [24].

Regulatory relationships of Rbpj

  • Nrarp overexpression is able to block Notch-induced activation of CBF-1 [25].
  • In addition, both ligands transmitted signal through the CBF-1-dependent pathway and stimulated the expression of HES-1, a direct target of Notch pathway [26].

Other interactions of Rbpj

  • Taken together, mouse Notch1 RAMIC can experimentally be separated into three functional domains: the RAM domain and ANK repeats for RBP-J binding and co-repressor displacement and the C-terminal TAD [15].
  • Loss of RBP-J and Pofut1 led to an accumulation of basal cell clusters characterized by the presence of cytokeratins (K5) and K14 and smooth muscle actin (SMA) during pregnancy [27].
  • The transcription factor recombination signal sequence-binding protein Jkappa (RBP-J) is a key downstream element in the signaling pathway of all four mammalian Notch receptors that are critically involved in the control of embryonic and adult development [16].
  • We have isolated a cDNA clone (RBP-2) for the protein (RBP-J kappa) which binds to immunoglobulin recombination signals with 23-base pair spacers (Matsunami, N., Hamaguchi, Y., Yamamoto, Y., Kuze, K., Kangawa, K., Matsuo, H., Kawaichi, M., and Honjo, T. (1989) Nature 342, 934-937) [28].
  • The DNA binding activity was not affected by amino acid replacements within the integrase motif of the RBP-J kappa protein (230His-269His) [29].

Analytical, diagnostic and therapeutic context of Rbpj

  • Site-directed mutagenesis study on DNA binding regions of the mouse homologue of Suppressor of Hairless, RBP-J kappa [29].
  • Several lines of evidence indicated that partial denervation was rare in old CBF-1 mice, and therefore could not account for the findings above [30].
  • We have investigated whether J kappa recombination signal sequence (RS) binding protein (RBP-J kappa) has any partial catalytic activities involved in the VDJ recombination reaction, such as cleavage, ligation, and bending of DNA [4].


  1. Notch/RBP-J signaling regulates epidermis/hair fate determination of hair follicular stem cells. Yamamoto, N., Tanigaki, K., Han, H., Hiai, H., Honjo, T. Curr. Biol. (2003) [Pubmed]
  2. Effects of adult F1 thymocytes on graft-versus-host splenomegaly and homing of parental normal and cortisone-resistant thymocytes in F1 neonates. Pierce, G.E., Clancy, J. Transplantation (1980) [Pubmed]
  3. Murine gammaherpesvirus 68 Rta-dependent activation of the gene 57 promoter. Pavlova, I., Lin, C.Y., Speck, S.H. Virology (2005) [Pubmed]
  4. Biochemical and immunological characterization of the DNA binding protein (RBP-J kappa) to mouse J kappa recombination signal sequence. Hamaguchi, Y., Yamamoto, Y., Iwanari, H., Maruyama, S., Furukawa, T., Matsunami, N., Honjo, T. J. Biochem. (1992) [Pubmed]
  5. Nitric oxide increases the amount of murine cytomegalovirus-DNA in mice latently infected with the virus. Okada, K., Tanaka, K., Noda, S., Okazaki, M., Koga, Y. Arch. Virol. (1999) [Pubmed]
  6. Learning and memory deficits in Notch mutant mice. Costa, R.M., Honjo, T., Silva, A.J. Curr. Biol. (2003) [Pubmed]
  7. Haploinsufficient lethality and formation of arteriovenous malformations in Notch pathway mutants. Krebs, L.T., Shutter, J.R., Tanigaki, K., Honjo, T., Stark, K.L., Gridley, T. Genes Dev. (2004) [Pubmed]
  8. Notch signaling regulates left-right asymmetry determination by inducing Nodal expression. Krebs, L.T., Iwai, N., Nonaka, S., Welsh, I.C., Lan, Y., Jiang, R., Saijoh, Y., O'Brien, T.P., Hamada, H., Gridley, T. Genes Dev. (2003) [Pubmed]
  9. Regulation of alphabeta/gammadelta T cell lineage commitment and peripheral T cell responses by Notch/RBP-J signaling. Tanigaki, K., Tsuji, M., Yamamoto, N., Han, H., Tsukada, J., Inoue, H., Kubo, M., Honjo, T. Immunity (2004) [Pubmed]
  10. Regulation of marginal zone B cell development by MINT, a suppressor of Notch/RBP-J signaling pathway. Kuroda, K., Han, H., Tani, S., Tanigaki, K., Tun, T., Furukawa, T., Taniguchi, Y., Kurooka, H., Hamada, Y., Toyokuni, S., Honjo, T. Immunity (2003) [Pubmed]
  11. Notch-RBP-J signaling is involved in cell fate determination of marginal zone B cells. Tanigaki, K., Han, H., Yamamoto, N., Tashiro, K., Ikegawa, M., Kuroda, K., Suzuki, A., Nakano, T., Honjo, T. Nat. Immunol. (2002) [Pubmed]
  12. The effects of fexofenadine on eosinophilia and systemic anaphylaxis in mice infected with Trichinella spiralis. Watanabe, N., Matsuda, E., Masuda, A., Nariai, K., Shibasaki, T. Int. Immunopharmacol. (2004) [Pubmed]
  13. Assignment of the mouse Rbpsuh gene to chromosome 5 and one processed pseudogene Rbpsuh-rs3 to chromosome 6. Klein, B.S., Himmelbauer, H., Zechner, U., Riemann, M., Liptay, S., Hameister, H., Schmid, R.M. Cytogenet. Cell Genet. (2000) [Pubmed]
  14. Disruption of the mouse RBP-J kappa gene results in early embryonic death. Oka, C., Nakano, T., Wakeham, A., de la Pompa, J.L., Mori, C., Sakai, T., Okazaki, S., Kawaichi, M., Shiota, K., Mak, T.W., Honjo, T. Development (1995) [Pubmed]
  15. Roles of the ankyrin repeats and C-terminal region of the mouse notch1 intracellular region. Kurooka, H., Kuroda, K., Honjo, T. Nucleic Acids Res. (1998) [Pubmed]
  16. Recombination signal sequence-binding protein Jkappa alters mesodermal cell fate decisions by suppressing cardiomyogenesis. Schroeder, T., Fraser, S.T., Ogawa, M., Nishikawa, S., Oka, C., Bornkamm, G.W., Nishikawa, S., Honjo, T., Just, U. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  17. RBP-Jkappa-dependent notch signaling is dispensable for mouse early embryonic development. Souilhol, C., Cormier, S., Tanigaki, K., Babinet, C., Cohen-Tannoudji, M. Mol. Cell. Biol. (2006) [Pubmed]
  18. Inhibition of Notch/RBP-J signaling induces hair cell formation in neonate mouse cochleas. Yamamoto, N., Tanigaki, K., Tsuji, M., Yabe, D., Ito, J., Honjo, T. J. Mol. Med. (2006) [Pubmed]
  19. Inducible gene knockout of transcription factor recombination signal binding protein-J reveals its essential role in T versus B lineage decision. Han, H., Tanigaki, K., Yamamoto, N., Kuroda, K., Yoshimoto, M., Nakahata, T., Ikuta, K., Honjo, T. Int. Immunol. (2002) [Pubmed]
  20. Synthesis of two classes of antibody, gammaM and gammaG or gammaM and gammaA, by identical cells. Amplification of the antibody response to pneumococcal polysaccharide type III. Pasanen, V.J., Asofsky, R., Baker, P.J. J. Exp. Med. (1979) [Pubmed]
  21. Metabolism of choline in brain of the aged CBF-1 mouse. Saito, M., Kindel, G., Karczmar, A.G., Rosenberg, A. J. Neurosci. Res. (1986) [Pubmed]
  22. Progression of age changes in mature mouse motor nerve terminals and its relation to locomotor activity. Robbins, N., Fahim, M.A. J. Neurocytol. (1985) [Pubmed]
  23. Early pancreatic development requires the vertebrate Suppressor of Hairless (RBPJ) in the PTF1 bHLH complex. Masui, T., Long, Q., Beres, T.M., Magnuson, M.A., MacDonald, R.J. Genes Dev. (2007) [Pubmed]
  24. A nonclassical bHLH Rbpj transcription factor complex is required for specification of GABAergic neurons independent of Notch signaling. Hori, K., Cholewa-Waclaw, J., Nakada, Y., Glasgow, S.M., Masui, T., Henke, R.M., Wildner, H., Martarelli, B., Beres, T.M., Epstein, J.A., Magnuson, M.A., Macdonald, R.J., Birchmeier, C., Johnson, J.E. Genes Dev. (2008) [Pubmed]
  25. Notch-regulated ankyrin-repeat protein inhibits Notch1 signaling: multiple Notch1 signaling pathways involved in T cell development. Yun, T.J., Bevan, M.J. J. Immunol. (2003) [Pubmed]
  26. Critical regulation of bone morphogenetic protein-induced osteoblastic differentiation by Delta1/Jagged1-activated Notch1 signaling. Nobta, M., Tsukazaki, T., Shibata, Y., Xin, C., Moriishi, T., Sakano, S., Shindo, H., Yamaguchi, A. J. Biol. Chem. (2005) [Pubmed]
  27. The canonical Notch/RBP-J signaling pathway controls the balance of cell lineages in mammary epithelium during pregnancy. Buono, K.D., Robinson, G.W., Martin, C., Shi, S., Stanley, P., Tanigaki, K., Honjo, T., Hennighausen, L. Dev. Biol. (2006) [Pubmed]
  28. Genomic organization of mouse J kappa recombination signal binding protein (RBP-J kappa) gene. Kawaichi, M., Oka, C., Shibayama, S., Koromilas, A.E., Matsunami, N., Hamaguchi, Y., Honjo, T. J. Biol. Chem. (1992) [Pubmed]
  29. Site-directed mutagenesis study on DNA binding regions of the mouse homologue of Suppressor of Hairless, RBP-J kappa. Chung, C.N., Hamaguchi, Y., Honjo, T., Kawaichi, M. Nucleic Acids Res. (1994) [Pubmed]
  30. Neural cell adhesion molecule in aged mouse muscle. Kobayashi, H., Robbins, N., Rutishauser, U. Neuroscience (1992) [Pubmed]
WikiGenes - Universities