Nuove strategie farmacologiche per la sindrome di Marfan: dai modelli animali all'uomo [Novel pharmacological strategies for aortic dilation in Marfan syndrome: from mouse models to human patients]

Item Type:	Article
Title:	Nuove strategie farmacologiche per la sindrome di Marfan: dai modelli animali all'uomo [Novel pharmacological strategies for aortic dilation in Marfan syndrome: from mouse models to human patients]
Creators Name:	Perrino, C. and Schiattarella, G.G. and Bottino, R. and Magliulo, F. and Oliveti, M. and Esposito, G. and Trimarco, B.
Abstract:	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 transforming growth factor-β and angiotensin II receptor type 1 antagonists have been elucidated. This review focuses on the most commonly used animal models to investigate the molecular mechanisms underlying MS, and on novel pharmacological strategies to reduce aortic dilation in MS.
Keywords:	Aortic Aneurysm, Fibrillin-1, Losartan, Marfan Syndrome, Transforming Growth Factor-β, Animals, Mice
Source:	Giornale Italiano di Cardiologia
ISSN:	1827-6806
Publisher:	Federazione Italiana di Cardiologia
Volume:	15
Number:	7
Page Range:	408-417
Date:	July 2014
Official Publication:	https://doi.org/10.1714/1596.17416
PubMed:	View item in PubMed

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