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Am J Physiol Cell Physiol 293: C1-C11, 2007. First published March 21, 2007; doi:10.1152/ajpcell.00415.2006
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INVITED REVIEW

Notch signaling in the developing cardiovascular system

Kyle Niessen1,3 and Aly Karsan1,2,3,4

1Departments of Medical Biophysics and 2Pathology and Laboratory Medicine, British Columbia Cancer Agency, Vancouver; and the 3Experimental Medicine Program and 4Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada

The Notch proteins encompass a family of transmembrane receptors that have been highly conserved through evolution as mediators of cell fate. Recent findings have demonstrated a critical role of Notch in the developing cardiovascular system. Notch signaling has been implicated in the endothelial-to-mesenchymal transition during development of the heart valves, in arterial-venous differentiation, and in remodeling of the primitive vascular plexus. Mutations of Notch pathway components in humans are associated with congenital defects of the cardiovascular system such as Alagille syndrome, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), and bicuspid aortic valves. This article focuses on the role of the Notch pathway in the developing cardiovascular system and congenital human cardiovascular diseases.

cardiac development; endothelial-mesenchymal transformation; vasculogenesis; angiogenesis


Address for reprint requests and other correspondence: A. Karsan, Dept. of Medical Biophysics, British Columbia Cancer Research Centre, 675 W. 10th Ave., Vancouver, BC, Canada V5Z 1L3 (e-mail: akarsan{at}bccrc.ca)




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