Comparative meta-analysis of cystic fibrosis cell models suggests partial endothelial-to-mesenchymal transition

Cystic fibrosis (CF) is a rare progressive disease, caused by bi-allelic pathogenic variants of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF is characterized by defective pulmonary mucociliary clearance leading to protracted inflammation and subsequent lung damage. Such inflammatory-driven lung damage is a well-known inducer of epithelial-to-mesenchymal transition (EMT), which can in turn lead to fibrosis, a disease mechanism of several lung diseases [1]. As such, transcriptomic meta-analysis has demonstrated that EMT, injury and inflammation were shared features between CF airway epithelium and other respiratory pathologies [2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Short communication Source Type: research