Article
[Novel pharmacological strategies for aortic dilation in Marfan syndrome: from mouse models to human patients].
Giornale italiano di cardiologia (2006) - 1 Jan 2000
Perrino Cinzia, Schiattarella Gabriele Giacomo, Bottino Roberta, Magliulo Fabio, Oliveti Marco, Esposito Giovanni, Trimarco Bruno
Abstract excerpt
Marfan syndrome (MS) is a congenital disorder of the connective tissue characterized by aortic dilation with frequent progression to aortic aneurysms requiring surgical intervention. Although mutations in the fibrillin-1 (FBN1) gene have been recognized as the genetic cause of MS a long time ago, only recently deeper knowledge of the molecular mechanisms underlying fibrillin-1 biology and the crucial role of...
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