The role of transforming growth factor-beta in Marfan syndrome

Autorzy: Kálmán Benke¹, Bence Ágg², Bálint Szilveszter², Ferenc Tarr², Zsolt B. Nagy³, Miklós Pólos², László Daróczi², Béla Merkely², Zoltán Szabolcs¹ 1 - Department of Cardiology and Cardiovascular Surgery, Semmelweis University, Budapest, Hungary, Hungarian Marfan Foundation, Budapest, Hungary
2 - Department of Cardiology and Cardiovascular Surgery, Semmelweis University, Budapest, Hungary
3 - Genetics for Health Association, Budapest, Hungary

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Abstrakt

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 on the tissue homeostasis of microfi brils, and elastic fi bers. We also investigate current data on the extracellular regulation of TGF-b level including mechanotransduction and the feedback cycles of integrin-dependent and independent activation of the latent TGF-b complex. Together these factors, by the destruction of the connective tissue fi bers, may play an important role in the 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 the treatment of the syndrome, and explore possible avenues of research into pathogenesis of MFS in order to improve understanding of the disease.

Wydane przez: Via Medica
Data wydania: 2013-06-10
Język: angielski
Profil: choroby sercowo-naczyniowe

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

Rodzaj: Artykuł, Dostęp: Dla wszystkich, Odpłatność: Darmowe