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

ABSTRACT:

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 inherited deficiency of connective tissue might play a role in the combined development of pulmonary emphysema and vascular disease.

Methods

We first determined the prevalence of chronic obstructive pulmonary disease in a clinical cohort of aortic aneurysms patients and arterial occlusive disease patients. Subsequently, we used a combined approach comprising pathological, functional, molecular imaging, immunological and gene expression analysis to reveal the sequence of events that culminates in pulmonary emphysema in aneurysmal Fibulin-4 deficient (Fibulin-4(R)) mice.

Results

SUBMITTER: Ramnath NW 

PROVIDER: S-EPMC4177830 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature