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Am J Physiol Cell Physiol (March 21, 2007). doi:10.1152/ajpcell.00415.2006
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Submitted on August 3, 2006
Accepted on March 14, 2007

Notch Signaling in the Developing Cardiovascular System

Kyle Niessen1 and Aly Karsan1*

1 Medical Biophysics, BC Cancer Research Centre, Vancouver, Canada

* To whom correspondence should be addressed. E-mail: akarsan{at}bccrc.ca.

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 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.




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