Accordingly, it was shown that aortic endothelial cells from CAVD patients have a down-regulated Notch signaling state and fail to activate Notch-dependent endothelial-to-mesenchymal transition (EMT) when stimulated by different Notch ligands or transforming growth factor-β (TGF-β) [17]. This evidence concerns the gene TGFB1 and congenital bilateral aplasia of vas deferens from CFTR mutation.