Gene Symbol: Rbpj
Description: recombination signal binding protein for immunoglobulin kappa J region
Alias: recombining binding protein suppressor of hairless
Species: rat
Products:     Rbpj

Top Publications

  1. de la Pompa J, Wakeham A, Correia K, Samper E, Brown S, Aguilera R, et al. Conservation of the Notch signalling pathway in mammalian neurogenesis. Development. 1997;124:1139-48 pubmed
    ..Our results demonstrate conservation of the Notch pathway and its regulatory mechanisms from fly to mouse, and support a role for the murine Notch signalling pathway in the regulation of neural stem cell differentiation. ..
  2. del Monte G, Grego Bessa J, González Rajal A, Bolós V, de la Pompa J. Monitoring Notch1 activity in development: evidence for a feedback regulatory loop. Dev Dyn. 2007;236:2594-614 pubmed
    ..We found that Notch1 transcription and activity was severely reduced in zebrafish and mouse Notch pathway mutants, suggesting that vertebrate Notch1 expression is regulated by a positive feedback loop. ..
  3. Oswald F, Winkler M, Cao Y, Astrahantseff K, Bourteele S, Knöchel W, et al. RBP-Jkappa/SHARP recruits CtIP/CtBP corepressors to silence Notch target genes. Mol Cell Biol. 2005;25:10379-90 pubmed
    ..Furthermore, the endogenous Hey1 promoter is derepressed in CtBP-deficient cells. We propose that a corepressor complex containing CtIP/CtBP facilitates RBP-Jkappa/SHARP-mediated repression of Notch target genes. ..
  4. Nickenig G, Wolff M, Stablein A, Pfister H, Bohm M. Enhanced expression and autoimmunity of recombination signal binding protein-jkappa in human dilated cardiomyopathy. Biochem Biophys Res Commun. 1999;266:432-6 pubmed
    ..In this setting, enhanced myocardial RBP-jkappa expression could represent a compensatory but ineffective response to counteract the increased rate of apoptosis in DCM. Furthermore, RBP-jkappa may be a useful diagnostic marker for DCM. ..
  5. Ma X, Renda M, Wang L, Cheng E, Niu C, Morris S, et al. Rbm15 modulates Notch-induced transcriptional activation and affects myeloid differentiation. Mol Cell Biol. 2007;27:3056-64 pubmed
    ..Thus, Rbm15 is differentially expressed during hematopoiesis and may act to inhibit myeloid differentiation in hematopoietic cells via a mechanism that is mediated by stimulation of Notch signaling via RBPJkappa. ..
  6. Nus M, MacGrogan D, Martínez Poveda B, Benito Y, Casanova J, Fernandez Aviles F, et al. Diet-induced aortic valve disease in mice haploinsufficient for the Notch pathway effector RBPJK/CSL. Arterioscler Thromb Vasc Biol. 2011;31:1580-8 pubmed publisher
    ..Our data establish a genetic mouse model of calcific aortic valve disease and may help to identify a patient population with reduced valvular NOTCH signaling at risk for developing this disease. ..
  7. Krebs L, Shutter J, Tanigaki K, Honjo T, Stark K, Gridley T. Haploinsufficient lethality and formation of arteriovenous malformations in Notch pathway mutants. Genes Dev. 2004;18:2469-73 pubmed
    ..These results demonstrate that vascular remodeling in the mouse embryo is sensitive to Dll4 gene dosage and that Notch activation in endothelial cells is essential for embryonic vascular remodeling. ..
  8. Wacker S, Alvarado C, von Wichert G, Knippschild U, Wiedenmann J, Clauss K, et al. RITA, a novel modulator of Notch signalling, acts via nuclear export of RBP-J. EMBO J. 2011;30:43-56 pubmed publisher
    ..Thus, we propose that RITA acts as a negative modulator of the Notch signalling pathway, controlling the level of nuclear RBP-J/CBF-1, where its amounts are limiting...
  9. Wall D, Mears A, McNeill B, Mazerolle C, Thurig S, Wang Y, et al. Progenitor cell proliferation in the retina is dependent on Notch-independent Sonic hedgehog/Hes1 activity. J Cell Biol. 2009;184:101-12 pubmed publisher
    ..This paper is the first demonstration of the mechanistic and functional link between Shh, Gli, and Hes1 in the regulation of progenitor cell behavior. ..

More Information


  1. Fang T, Yashiro Ohtani Y, Del Bianco C, Knoblock D, Blacklow S, Pear W. Notch directly regulates Gata3 expression during T helper 2 cell differentiation. Immunity. 2007;27:100-10 pubmed
    ..Together, these data implicate Gata3 as a direct transcriptional Notch target that acts in concert with Notch signaling to generate optimal Th2 cell responses. ..
  2. Yun T, Bevan M. Notch-regulated ankyrin-repeat protein inhibits Notch1 signaling: multiple Notch1 signaling pathways involved in T cell development. J Immunol. 2003;170:5834-41 pubmed
    ..The different effects of Deltex-1 and Nrarp overexpression suggest that alternate Notch signaling pathways mediate T vs B lineage commitment and thymocyte maturation. ..
  3. Markova N, Pinkas Sarafova A, Simon M. A metabolic enzyme of the short-chain dehydrogenase/reductase superfamily may moonlight in the nucleus as a repressor of promoter activity. J Invest Dermatol. 2006;126:2019-31 pubmed
    ..These findings add hRoDH-E2 to the small group of metabolic enzymes, which, by being recruited over promoter regions, could directly link the cytoplasmic and nuclear functions within the cell. ..
  4. Hsieh J, Zhou S, Chen L, Young D, Hayward S. CIR, a corepressor linking the DNA binding factor CBF1 to the histone deacetylase complex. Proc Natl Acad Sci U S A. 1999;96:23-8 pubmed
    ..When expressed as a Gal4 fusion protein, CIR repressed reporter gene expression. CIR binds to histone deacetylase and to SAP30 and serves as a linker between CBF1 and the histone deacetylase complex. ..
  5. Garcia T, Farmaha J, Kow S, Hofmann M. RBPJ in mouse Sertoli cells is required for proper regulation of the testis stem cell niche. Development. 2014;141:4468-78 pubmed publisher
    ..To this end, we conditionally ablated recombination signal binding protein for immunoglobulin kappa J region (Rbpj), a crucial mediator of NOTCH signaling, in Sertoli cells using Amh-cre...
  6. Guha A, Vasconcelos M, Cai Y, Yoneda M, Hinds A, Qian J, et al. Neuroepithelial body microenvironment is a niche for a distinct subset of Clara-like precursors in the developing airways. Proc Natl Acad Sci U S A. 2012;109:12592-7 pubmed publisher
  7. Ferjentsik Z, Hayashi S, Dale J, Bessho Y, Herreman A, De Strooper B, et al. Notch is a critical component of the mouse somitogenesis oscillator and is essential for the formation of the somites. PLoS Genet. 2009;5:e1000662 pubmed publisher
    ..embryos carrying a mutation in different components of the Notch pathway, such as Lunatic fringe (Lfng), Hes7, Rbpj, and presenilin1/presenilin2 (Psen1/Psen2), and by pharmacological blocking of the Notch pathway...
  8. Minoguchi S, Taniguchi Y, Kato H, Okazaki T, Strobl L, Zimber Strobl U, et al. RBP-L, a transcription factor related to RBP-Jkappa. Mol Cell Biol. 1997;17:2679-87 pubmed
    ..This is again in contrast to physical association of RBP-J with EBNA-2. Several models to explain functional interaction between RBP-L and EBNA-2 are discussed. ..
  9. Robert Moreno A, Espinosa L, de la Pompa J, Bigas A. RBPjkappa-dependent Notch function regulates Gata2 and is essential for the formation of intra-embryonic hematopoietic cells. Development. 2005;132:1117-26 pubmed
    ..Taken together, these data strongly suggest that activation of Gata2 expression by Notch1/RBPjkappa is a crucial event for the onset of definitive hematopoiesis in the embryo. ..
  10. Sweeney C, Morrow D, Birney Y, Coyle S, Hennessy C, Scheller A, et al. Notch 1 and 3 receptor signaling modulates vascular smooth muscle cell growth, apoptosis, and migration via a CBF-1/RBP-Jk dependent pathway. FASEB J. 2004;18:1421-3 pubmed
    ..Notch signaling, therefore, represents a novel therapeutic target for disease states in which changes in vascular cell fate occur in vivo. ..
  11. Doetzlhofer A, Basch M, Ohyama T, Gessler M, Groves A, Segil N. Hey2 regulation by FGF provides a Notch-independent mechanism for maintaining pillar cell fate in the organ of Corti. Dev Cell. 2009;16:58-69 pubmed publisher
  12. Krebs L, Iwai N, Nonaka S, Welsh I, Lan Y, Jiang R, et al. Notch signaling regulates left-right asymmetry determination by inducing Nodal expression. Genes Dev. 2003;17:1207-12 pubmed
    ..Our results demonstrate that Dll1-mediated Notch signaling is essential for generation of left-right asymmetry, and that the Notch pathway acts upstream of Nodal expression during left-right asymmetry determination in mice. ..
  13. Masui T, Long Q, Beres T, Magnuson M, Macdonald R. Early pancreatic development requires the vertebrate Suppressor of Hairless (RBPJ) in the PTF1 bHLH complex. Genes Dev. 2007;21:2629-43 pubmed
    ..We show that early in pancreatic development, active PTF1a requires interaction with RBPJ, the vertebrate Suppressor of Hairless, within a stable trimeric DNA-binding complex (PTF1)...
  14. Niessen K, Fu Y, Chang L, Hoodless P, McFadden D, Karsan A. Slug is a direct Notch target required for initiation of cardiac cushion cellularization. J Cell Biol. 2008;182:315-25 pubmed publisher
    ..Collectively, our data suggest that combined expression of Slug and Snail is required for EMT in cardiac cushion morphogenesis. ..
  15. Shen H, McElhinny A, Cao Y, Gao P, Liu J, Bronson R, et al. The Notch coactivator, MAML1, functions as a novel coactivator for MEF2C-mediated transcription and is required for normal myogenesis. Genes Dev. 2006;20:675-88 pubmed
    ..Mechanistically, MAML1 appears to mediate cross-talk between Notch and MEF2 to influence myogenic differentiation. ..
  16. Issack P, Ziff E. Genetic elements regulating HES-1 induction in Wnt-1-transformed PC12 cells. Cell Growth Differ. 1998;9:827-36 pubmed
    ..One element binds Wnt-1-induced protein complexes in a sequence-specific manner. Identification of Wnt-1 responsive elements in potential target genes may provide clues to nuclear pathways regulated by Wnt-1. ..
  17. Satpathy A, Briseño C, Lee J, Ng D, Manieri N, Kc W, et al. Notch2-dependent classical dendritic cells orchestrate intestinal immunity to attaching-and-effacing bacterial pathogens. Nat Immunol. 2013;14:937-48 pubmed publisher
    ..rodentium, but CD103(+) cDCs dependent on the transcription factor Batf3 were not. Our results demonstrate a nonredundant function for CD11b(+) cDCs in the response to pathogens in vivo. ..
  18. Levy O, Lah J, Levey A. Notch signaling inhibits PC12 cell neurite outgrowth via RBP-J-dependent and -independent mechanisms. Dev Neurosci. 2002;24:79-88 pubmed
    ..Conversely, an activated form of RBP-J decreased neurite length but failed to reduce neurite number. In summary, Notch activation inhibited PC12 cell neurite outgrowth by both RBP-J-dependent and -independent pathways. ..
  19. Tanigaki K, Han H, Yamamoto N, Tashiro K, Ikegawa M, Kuroda K, et al. Notch-RBP-J signaling is involved in cell fate determination of marginal zone B cells. Nat Immunol. 2002;3:443-50 pubmed
    ..In contrast, these mice exhibited increased mortality rates after blood-borne bacterial infection, which indicates that MZB cells play pivotal roles in the clearance of these bacteria. ..
  20. Lu J, Zhou Y, Hu T, Zhang H, Shen M, Cheng P, et al. Notch Signaling Coordinates Progenitor Cell-Mediated Biliary Regeneration Following Partial Hepatectomy. Sci Rep. 2016;6:22754 pubmed publisher
    ..Although the transcription factor RBPJ is essential for liver morphogenesis and biliary development, its specific function in the differentiation of ..
  21. Jeffries S, Robbins D, Capobianco A. Characterization of a high-molecular-weight Notch complex in the nucleus of Notch(ic)-transformed RKE cells and in a human T-cell leukemia cell line. Mol Cell Biol. 2002;22:3927-41 pubmed
    ..3) translocation and constitutively express N(ic). Taken together, our data indicate that complex formation is likely required for neoplastic transformation by Notch(ic)...
  22. Apelqvist A, Li H, Sommer L, Beatus P, Anderson D, Honjo T, et al. Notch signalling controls pancreatic cell differentiation. Nature. 1999;400:877-81 pubmed
    ..These data provide evidence that ngn3 acts as proendocrine gene and that Notch signalling is critical for the decision between the endocrine and progenitor/exocrine fates in the developing pancreas. ..
  23. Dai Q, Andreu Agullo C, Insolera R, Wong L, Shi S, Lai E. BEND6 is a nuclear antagonist of Notch signaling during self-renewal of neural stem cells. Development. 2013;140:1892-902 pubmed publisher
    ..These data reveal a novel mammalian CSL co-repressor in the nervous system, and show that the Notch-inhibitory activity of certain BEN-solo proteins is conserved between flies and mammals. ..
  24. Chang J, Meredith D, Mayer P, Borromeo M, Lai H, Ou Y, et al. Prdm13 mediates the balance of inhibitory and excitatory neurons in somatosensory circuits. Dev Cell. 2013;25:182-95 pubmed publisher
    ..Thus, Prdm13 is a key component of a highly coordinated transcriptional network that determines the balance of inhibitory versus excitatory neurons in the dorsal spinal cord. ..
  25. Zhou S, Fujimuro M, Hsieh J, Chen L, Miyamoto A, Weinmaster G, et al. SKIP, a CBF1-associated protein, interacts with the ankyrin repeat domain of NotchIC To facilitate NotchIC function. Mol Cell Biol. 2000;20:2400-10 pubmed
    ..The results suggest a model in which NotchIC activates responsive promoters by competing with the SMRT-corepressor complex for contacts on both CBF1 and SKIP. ..
  26. Aras S, Pak O, Sommer N, FINLEY R, Huttemann M, Weissmann N, et al. Oxygen-dependent expression of cytochrome c oxidase subunit 4-2 gene expression is mediated by transcription factors RBPJ, CXXC5 and CHCHD2. Nucleic Acids Res. 2013;41:2255-66 pubmed publisher
    ..three transcription factors that bind this conserved ORE, namely recombination signal sequence-binding protein J? (RBPJ), coiled-coil-helix-coiled-coil-helix domain 2 (CHCHD2) and CXXC finger protein 5 (CXXC5)...
  27. Lee S, Wang X, DeJong J. Functional interactions between an atypical NF-kappaB site from the rat CYP2B1 promoter and the transcriptional repressor RBP-Jkappa/CBF1. Nucleic Acids Res. 2000;28:2091-8 pubmed
    ..The results suggest that RBP-Jkappa/CBF1 binds an atypical NF-kappaB site in the CYP2B1/2 promoters and may help to maintain a low level of expression in the absence of inducer. ..
  28. Amsen D, Antov A, Jankovic D, Sher A, Radtke F, Souabni A, et al. Direct regulation of Gata3 expression determines the T helper differentiation potential of Notch. Immunity. 2007;27:89-99 pubmed
    ..Moreover, absence of GATA-3 turned Notch from a Th2 inducer into a powerful inducer of Th1 differentiation. Therefore, Gata3 is a critical element determining inductive Th2 differentiation and limiting Th1 differentiation by Notch. ..
  29. Yoon K, Nery S, Rutlin M, Radtke F, Fishell G, Gaiano N. Fibroblast growth factor receptor signaling promotes radial glial identity and interacts with Notch1 signaling in telencephalic progenitors. J Neurosci. 2004;24:9497-506 pubmed