Abstract:Background
Aortic dissection and rupture is the main cause of early cardiovascular mortality in patients with Marfan syndrome (MFS). MFS is caused by a defect in fibrillin-1, a building block of microfibrils in the extracellular matrix which binds transforming growth factor beta (TGF-beta) via interaction with latent TGF-beta binding proteins (LTBPs). Multiple mouse models, both pharmaceutically induced and genetically manipulated, have been used to investigate the pathophysiology and biomech… Show more
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