Key result
Endocardial deletion of Jag1, Notch1, or RBPJ in mice caused enlarged valve cusps, bicuspid aortic valve, and septal defects by failing to restrict Bmp-mediated mesenchyme proliferation.
This study identifies that sequential Dll4-Notch1 and Jag1-Notch1 signaling regulates cardiac valve formation and morphogenesis, providing a molecular mechanism for congenital heart defects like bicuspid aortic valve associated with reduced NOTCH function.
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Endocardial Notch loss enlarges cusps in mice; leaves open relevance to human bicuspid aortic valve and septal defects.
MacGrogan et al. (2016) studied Congenital heart defects and valve dysmorphology. Endothelial/endocardial deletion of Notch signaling components (Mib1-Dll4-Notch1, Jag1, RBPJ) was evaluated on Cardiac epithelial-mesenchymal transition and valvulogenesis. Endocardial deletion of Jag1, Notch1, or RBPJ in mice caused enlarged valve cusps, bicuspid aortic valve, and septal defects by failing to restrict Bmp-mediated mesenchyme proliferation.
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