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Gene Review

Notch3  -  notch 3

Mus musculus

Synonyms: AW229011, N3, Neurogenic locus notch homolog protein 3, Notch 3, hpbk
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Disease relevance of Notch3


Psychiatry related information on Notch3

  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized adult-onset autosomal dominant vascular dementia, caused by highly stereotyped mutations in the Notch3 receptor [5].

High impact information on Notch3


Chemical compound and disease context of Notch3


Biological context of Notch3


Anatomical context of Notch3

  • Notch2, Notch3, and Dll1 are initially expressed by most cells within the pituitary primordium and become restricted to a subset of the progenitor cell pool as differentiated pituitary cells begin to appear [12].
  • Therefore, the thymus might be a target tissue affected by Notch3 deficiency [9].
  • In the newborn mouse liver, Notch2 and Notch3 are expressed in opposing cell populations, suggesting they play different roles in cell fate determination during bile duct development [13].
  • Notch3, Dll-1 and Dtx1exhibit a stage dependent expression as their mRNAs are detected in 2-cell embryos and in hatched blastocysts, but are absent or weakly detected at the morula stage [14].
  • In addition we could detect Notch3 mRNA and protein in cell lines representing mast cell progenitors [11].

Associations of Notch3 with chemical compounds

  • Notch3 and 4 were not detected in the presence or absence of cortisol [15].
  • During regeneration of spermatogonia in busulfan-treated mice, the nuclei of all proliferating cells showed staining for the intracellular domain of Notch3 [16].
  • Here we describe the generation of a mouse knockin model for one of the most prevalent CADASIL mutations, an arginine to cysteine transition at position 141, R141C, which corresponds to mutation R142C in mouse NOTCH 3 [17].

Regulatory relationships of Notch3


Other interactions of Notch3

  • Notch1, Notch2 and Notch3 were immunohistochemically detected in non-neuroendocrine cells [20].
  • Moreover, a differential distribution of Notch3, with respect to Notch1, was observed in distinct age-related thymocyte subsets [21].
  • These results suggest distinct roles of Notch1 and Notch3 in the neurogenesis of the peripheral olfactory system [22].
  • Notch 3 and Jagged 1 were absent from developing articular cartilage but were present in deeper layers at later time points (> 1 month) and both receptor and ligand were expressed in hypertrophic chondrocytes at all ages examined [23].
  • There is also evidence that the mouse Notch4 gene is the result of a rapid divergence from a Notch3-like gene [24].

Analytical, diagnostic and therapeutic context of Notch3


  1. Long-lasting induction of Notch2 in the hippocampus of kainate-treated adult mice. Toninelli, G.F., Bernardi, C., Quarto, M., Lozza, G., Memo, M., Grilli, M. Neuroreport (2003) [Pubmed]
  2. Constitutive activation of NF-kappaB and T-cell leukemia/lymphoma in Notch3 transgenic mice. Bellavia, D., Campese, A.F., Alesse, E., Vacca, A., Felli, M.P., Balestri, A., Stoppacciaro, A., Tiveron, C., Tatangelo, L., Giovarelli, M., Gaetano, C., Ruco, L., Hoffman, E.S., Hayday, A.C., Lendahl, U., Frati, L., Gulino, A., Screpanti, I. EMBO J. (2000) [Pubmed]
  3. Expression of activated Notch3 in transgenic mice enhances generation of T regulatory cells and protects against experimental autoimmune diabetes. Anastasi, E., Campese, A.F., Bellavia, D., Bulotta, A., Balestri, A., Pascucci, M., Checquolo, S., Gradini, R., Lendahl, U., Frati, L., Gulino, A., Di Mario, U., Screpanti, I. J. Immunol. (2003) [Pubmed]
  4. Constitutive activation of Notch3 inhibits terminal epithelial differentiation in lungs of transgenic mice. Dang, T.P., Eichenberger, S., Gonzalez, A., Olson, S., Carbone, D.P. Oncogene (2003) [Pubmed]
  5. Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Ruchoux, M.M., Domenga, V., Brulin, P., Maciazek, J., Limol, S., Tournier-Lasserve, E., Joutel, A. Am. J. Pathol. (2003) [Pubmed]
  6. Notch3 is required for arterial identity and maturation of vascular smooth muscle cells. Domenga, V., Fardoux, P., Lacombe, P., Monet, M., Maciazek, J., Krebs, L.T., Klonjkowski, B., Berrou, E., Mericskay, M., Li, Z., Tournier-Lasserve, E., Gridley, T., Joutel, A. Genes Dev. (2004) [Pubmed]
  7. Delta1-Notch3 interactions bias the functional differentiation of activated CD4+ T cells. Maekawa, Y., Tsukumo, S., Chiba, S., Hirai, H., Hayashi, Y., Okada, H., Kishihara, K., Yasutomo, K. Immunity (2003) [Pubmed]
  8. Notch3 and pre-TCR interaction unveils distinct NF-kappaB pathways in T-cell development and leukemia. Vacca, A., Felli, M.P., Palermo, R., Di Mario, G., Calce, A., Di Giovine, M., Frati, L., Gulino, A., Screpanti, I. EMBO J. (2006) [Pubmed]
  9. Functional redundancy of the Notch gene family during mouse embryogenesis: analysis of Notch gene expression in Notch3-deficient mice. Kitamoto, T., Takahashi, K., Takimoto, H., Tomizuka, K., Hayasaka, M., Tabira, T., Hanaoka, K. Biochem. Biophys. Res. Commun. (2005) [Pubmed]
  10. Active form of Notch members can enforce T lymphopoiesis on lymphoid progenitors in the monolayer culture specific for B cell development. Hozumi, K., Abe, N., Chiba, S., Hirai, H., Habu, S. J. Immunol. (2003) [Pubmed]
  11. Distinct and regulated expression of Notch receptors in hematopoietic lineages and during myeloid differentiation. Jönsson, J.I., Xiang, Z., Pettersson, M., Lardelli, M., Nilsson, G. Eur. J. Immunol. (2001) [Pubmed]
  12. Developmental regulation of Notch signaling genes in the embryonic pituitary: Prop1 deficiency affects Notch2 expression. Raetzman, L.T., Ross, S.A., Cook, S., Dunwoodie, S.L., Camper, S.A., Thomas, P.Q. Dev. Biol. (2004) [Pubmed]
  13. Characterization of Notch receptor expression in the developing mammalian heart and liver. Loomes, K.M., Taichman, D.B., Glover, C.L., Williams, P.T., Markowitz, J.E., Piccoli, D.A., Baldwin, H.S., Oakey, R.J. Am. J. Med. Genet. (2002) [Pubmed]
  14. Developmental expression of the Notch signaling pathway genes during mouse preimplantation development. Cormier, S., Vandormael-Pournin, S., Babinet, C., Cohen-Tannoudji, M. Gene Expr. Patterns (2004) [Pubmed]
  15. Cortisol regulates the expression of Notch in osteoblasts. Pereira, R.M., Delany, A.M., Durant, D., Canalis, E. J. Cell. Biochem. (2002) [Pubmed]
  16. Sequential activation of Notch family receptors during mouse spermatogenesis. Mori, S., Kadokawa, Y., Hoshinaga, K., Marunouchi, T. Dev. Growth Differ. (2003) [Pubmed]
  17. Mice carrying a R142C Notch 3 knock-in mutation do not develop a CADASIL-like phenotype. Lundkvist, J., Zhu, S., Hansson, E.M., Schweinhardt, P., Miao, Q., Beatus, P., Dannaeus, K., Karlström, H., Johansson, C.B., Viitanen, M., Rozell, B., Spenger, C., Mohammed, A., Kalimo, H., Lendahl, U. Genesis (2005) [Pubmed]
  18. Pre-TCR-triggered ERK signalling-dependent downregulation of E2A activity in Notch3-induced T-cell lymphoma. Talora, C., Campese, A.F., Bellavia, D., Pascucci, M., Checquolo, S., Groppioni, M., Frati, L., von Boehmer, H., Gulino, A., Screpanti, I. EMBO Rep. (2003) [Pubmed]
  19. Complementary and combinatorial patterns of Notch gene family expression during early mouse development. Williams, R., Lendahl, U., Lardelli, M. Mech. Dev. (1995) [Pubmed]
  20. Basic helix-loop-helix transcription factors regulate the neuroendocrine differentiation of fetal mouse pulmonary epithelium. Ito, T., Udaka, N., Yazawa, T., Okudela, K., Hayashi, H., Sudo, T., Guillemot, F., Kageyama, R., Kitamura, H. Development (2000) [Pubmed]
  21. Expression pattern of notch1, 2 and 3 and Jagged1 and 2 in lymphoid and stromal thymus components: distinct ligand-receptor interactions in intrathymic T cell development. Felli, M.P., Maroder, M., Mitsiadis, T.A., Campese, A.F., Bellavia, D., Vacca, A., Mann, R.S., Frati, L., Lendahl, U., Gulino, A., Screpanti, I. Int. Immunol. (1999) [Pubmed]
  22. Notch expression in developing olfactory neuroepithelium. Doi, K., Ishida, H., Nibu, K. Neuroreport (2004) [Pubmed]
  23. The distribution of Notch receptors and their ligands during articular cartilage development. Hayes, A.J., Dowthwaite, G.P., Webster, S.V., Archer, C.W. J. Anat. (2003) [Pubmed]
  24. Evolutionary analysis of vertebrate Notch genes. Kortschak, R.D., Tamme, R., Lardelli, M. Dev. Genes Evol. (2001) [Pubmed]
  25. Characterization of Notch3-deficient mice: normal embryonic development and absence of genetic interactions with a Notch1 mutation. Krebs, L.T., Xue, Y., Norton, C.R., Sundberg, J.P., Beatus, P., Lendahl, U., Joutel, A., Gridley, T. Genesis (2003) [Pubmed]
  26. Notch pathway genes are expressed in mammalian ovarian follicles. Johnson, J., Espinoza, T., McGaughey, R.W., Rawls, A., Wilson-Rawls, J. Mech. Dev. (2001) [Pubmed]
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