Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall |
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| Authors: | Gianluca L Perrucci Erica Rurali Aoife Gowran Alessandro Pini Carlo Antona Roberto Chiesa Giulio Pompilio Patrizia Nigro |
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| Institution: | 1.Department of Clinical Sciences and Community Health,University of Milan,Milan,Italy;2.Unit of Vascular Biology and Regenerative Medicine,Centro Cardiologico Monzino-IRCCS,Milan,Italy;3.Department of Cardiology, Marfan Clinic?,“Luigi Sacco” University of Milan,Milan,Italy;4.Cardiovascular Surgery Department,“Luigi Sacco” University of Milan,Milan,Italy;5.FoRCardioLab,“Luigi Sacco” University of Milan,Milan,Italy;6.Department of Vascular Surgery, San Raffaele Scientific Institute Hospital,Vita-Salute University,Milan,Italy;7.Department of Cardiovascular Surgery,Centro Cardiologico Monzino-IRCCS,Milan,Italy |
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| Abstract: | Marfan syndrome (MFS) is a connective tissue disorder with multiple organ manifestations. The genetic cause of this syndrome is the mutation of the FBN1 gene, encoding the extracellular matrix (ECM) protein fibrillin-1. This genetic alteration leads to the degeneration of microfibril structures and ECM integrity in the tunica media of the aorta. Indeed, thoracic aortic aneurysm and dissection represent the leading cause of death in MFS patients. To date, the most effective treatment option for this pathology is the surgical substitution of the damaged aorta. To highlight novel therapeutic targets, we review the molecular mechanisms related to MFS etiology in vascular smooth muscle cells, the foremost cellular type involved in MFS pathogenesis. |
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