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Physiology and pathology of notch signalling system
Author(s) -
Bianchi Silvia,
Dotti Maria Teresa,
Federico Antonio
Publication year - 2006
Publication title -
journal of cellular physiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.529
H-Index - 174
eISSN - 1097-4652
pISSN - 0021-9541
DOI - 10.1002/jcp.20542
Subject(s) - notch signaling pathway , cadasil , notch proteins , biology , hes3 signaling axis , microbiology and biotechnology , phenotype , signal transduction , transmembrane protein , signalling , transcription factor , neuroscience , hedgehog signaling pathway , receptor , leukoencephalopathy , genetics , gene , pathology , medicine , disease
Notch proteins encode a family of transmembrane receptors that are part of a signalling transduction system known as Notch signalling, an extremely conserved and widely used mechanism regulating programs governing growth, apoptosis and differentiation in metazoans. Notch signalling begins when the Notch receptor binds ligands and ends when the Notch intracellular domain enters the nucleus and activates transcription of target genes. This core pathway is subjected to a wide array of regulatory influences and protein‐protein interactions and is correlated with other signalling pathway. This review will sumarize recent findings concerning the physiology and pathology of Notch signalling in vascular development and homeostasis. Moreover, the clinical phenotypes of Notch3 signalling system pathology will be described, with particular regard to CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy) for which the most recent pathogenetic hypotheses are reported. J. Cell. Physiol. 207: 300–308, 2006. © 2005 Wiley‐Liss, Inc.