Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall

被引：41

作者：

Perrucci, Gianluca L. ^{[1
,2
]}

Rurali, Erica ^{[2
]}

Gowran, Aoife ^{[2
]}

Pini, Alessandro ^{[3
]}

Antona, Carlo ^{[4
,5
]}

Chiesa, Roberto ^{[6
]}

Pompilio, Giulio ^{[1
,2
,7
]}

Nigro, Patrizia ^{[2
]}

机构：

[1] Univ Milan, Dept Clin Sci & Community Hlth, Via Festa Perdono 7, I-20122 Milan, Italy

[2] Ctr Cardiol Monzino IRCCS, Unit Vasc Biol & Regenerat Med, Via C Parea 4, I-20138 Milan, Italy

[3] Luigi Sacco Univ Milan, Marfan Clin, Dept Cardiol, Via GB Grassi 74, I-20157 Milan, Italy

[4] Luigi Sacco Univ Milan, Cardiovasc Surg Dept, Via GB Grassi 74, I-20157 Milan, Italy

[5] Luigi Sacco Univ Milan, FoRCardioLab, Via GB Grassi 74, I-20157 Milan, Italy

[6] Univ Vita Salute San Raffaele, San Raffaele Sci Inst Hosp, Dept Vasc Surg, Milan, Italy

[7] Ctr Cardiol Monzino IRCCS, Dept Cardiovasc Surg, Via C Parea 4, I-20138 Milan, Italy

来源：

CELLULAR AND MOLECULAR LIFE SCIENCES | 2017年 / 74卷 / 02期

关键词：

Angiotensin II; TGF-beta; MMPs; MicroRNA; Mechanotrasduction; GROWTH-FACTOR-BETA; ACTIVATED-RECEPTOR-GAMMA; MATRIX METALLOPROTEINASE-2 AND-9; ANGIOTENSIN-II; TGF-BETA; MOUSE MODEL; FIBRILLIN GENE; SMAD PATHWAY; EXPRESSION; LOSARTAN;

D O I：

10.1007/s00018-016-2324-9

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：267 / 277

页数：11

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