open access

Vol 20, No 3 (2013)
Review articles
Published online: 2013-06-01
Submitted: 2013-06-10
Accepted: 2013-06-10
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The role of transforming growth factor-beta in Marfan syndrome

Kálmán Benke, Bence Ágg, Bálint Szilveszter, Ferenc Tarr, Zsolt B. Nagy, Miklós Pólos, László Daróczi, Béla Merkely, Zoltán Szabolcs
DOI: 10.5603/CJ.2013.0066
·
Cardiol J 2013;20(3):227-234.

open access

Vol 20, No 3 (2013)
Review articles
Published online: 2013-06-01
Submitted: 2013-06-10
Accepted: 2013-06-10

Abstract

The starting point, in Marfan syndrome (MFS) appears to be the mutation of fi brillin-1 gene whose deconstructed protein product cannot bind transforming growth factor beta (TGF-b), leading to an increased TGF-b tissue level. The aim of this review is to review the already known features of the cellular signal transduction downstream to TGF-b and its impact onthe tissue homeostasis of microfibrils, and elastic fi bers. We also investigate current data onthe extracellular regulation of TGF-b level including mechanotransduction and the feedback cycles of integrin-dependent and independent activation of the latent TGF-b complex. Togetherthese factors, by the destruction of the connective tissue fi bers, may play an important role inthe development of the diverse cardiac and extracardiac manifestations of MFS and many of them could be a target of conservative treatment. We present currently investigated drugs for thetreatment of the syndrome, and explore possible avenues of research into pathogenesis of MFS in order to improve understanding of the disease.

Abstract

The starting point, in Marfan syndrome (MFS) appears to be the mutation of fi brillin-1 gene whose deconstructed protein product cannot bind transforming growth factor beta (TGF-b), leading to an increased TGF-b tissue level. The aim of this review is to review the already known features of the cellular signal transduction downstream to TGF-b and its impact onthe tissue homeostasis of microfibrils, and elastic fi bers. We also investigate current data onthe extracellular regulation of TGF-b level including mechanotransduction and the feedback cycles of integrin-dependent and independent activation of the latent TGF-b complex. Togetherthese factors, by the destruction of the connective tissue fi bers, may play an important role inthe development of the diverse cardiac and extracardiac manifestations of MFS and many of them could be a target of conservative treatment. We present currently investigated drugs for thetreatment of the syndrome, and explore possible avenues of research into pathogenesis of MFS in order to improve understanding of the disease.
Get Citation

Keywords

Marfan syndrome, aortic aneurysm, transforming growth factor- -beta (TGF-b), matrix-metalloprotease (MMP)

About this article
Title

The role of transforming growth factor-beta in Marfan syndrome

Journal

Cardiology Journal

Issue

Vol 20, No 3 (2013)

Pages

227-234

Published online

2013-06-01

DOI

10.5603/CJ.2013.0066

Bibliographic record

Cardiol J 2013;20(3):227-234.

Keywords

Marfan syndrome
aortic aneurysm
transforming growth factor- -beta (TGF-b)
matrix-metalloprotease (MMP)

Authors

Kálmán Benke
Bence Ágg
Bálint Szilveszter
Ferenc Tarr
Zsolt B. Nagy
Miklós Pólos
László Daróczi
Béla Merkely
Zoltán Szabolcs

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