Molecular Pathogenesis of Fibulin-4 Associated Aortic Aneurysms

__Abstract__ Aortic disease represents one of the major causes of morbidity and mortality in the industrialized society. Approximately 2% of all deaths are caused by aortic aneurysm and dissections [1]. Aneurysms are defined as a local widening of the artery with more than 50 percent of the normal diameter [2]. An aneurysm can result in a fatal aortic rupture or in an aortic dissection, which is a tear in the wall of the artery that causes blood to flow between the layers of the wall and forces the layers apart [3]. Dissections involving the ascending aorta, Stanford type A dissections, have a mortality rate of 1-2% per hour and should be immediately treated surgically [4], while dissections occurring in the descending aorta are usually treated medically and have an in-hospital mortality of about 10% [5]. However, an acute aortic dissect

Extracellular matrix defects in aneurysmal Fibulin-4 mice predispose to lung emphysema

Background: In this study we set out to investigate the clinically observed relationship between chronic obstructive pulmonary disease (COPD) and aortic aneurysms. We tested the hypothesis that an