The knowledge of the interaction between EF and TGF-β signaling confirms the connection demonstrated in humans through mutations in FBN1, TGFBR1 and TGFBR2 genes that are known to cause MFS and overlapping disorder as Loeys-Dietz syndrome (LDS), familiar thoracic aorta aneurysms and dissections (Giusti et al., 2016). Here, TGFB1 is linked to Loeys-Dietz syndrome.