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Publication : Notch3: from subtle structural differences to functional diversity.

First Author  Bellavia D Year  2008
Journal  Oncogene Volume  27
Issue  38 Pages  5092-8
PubMed ID  18758477 Mgi Jnum  J:140988
Mgi Id  MGI:3815222 Doi  10.1038/onc.2008.230
Citation  Bellavia D, et al. (2008) Notch3: from subtle structural differences to functional diversity. Oncogene 27(38):5092-8
abstractText  The Notch3 gene was identified, at the beginning of 90s, as the third mammalian Notch and was initially reported as being expressed in proliferating neuroepithelium. Since then, increasing evidence has demonstrated a number of structural and functional differences between Notch3 and both Notch1 and Notch2, which exhibit the highest structural similarity among the four mammalian Notch receptors. Possibly due to its more restricted tissue distribution, targeted deletion of murine Notch3 does not lead to embryonic lethality as is observed with targeted deletion of Notch1 and Notch2. However, genetic mutation, amplification and deregulated expression of Notch3 have been correlated with the disruption of cell differentiation in transgenic mice and to development of diseases in mice and humans. This review discusses the possible relationships between the structural differences and the nonredundant roles that Notch3 plays in the pathogenesis of the human disease cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy and in the regulation of murine T-cell differentiation and leukemogenesis.
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