Mechanisms of endothelial cell dysfunction in cystic fibrosis
Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions of the cystic fibrosis
conductance regulator (CFTR) in vascular endothelial cells (EC) are poorly defined. We sought to uncover
biological activities of endothelial CFTR, relevant for vascular homeostasis and inflammation. We examined cells
from human umbilical cords (HUVEC) and pulmonary artery isolated from non-cystic fibrosis (PAEC) and CF
human lungs (CF-PAEC), under static conditions or physiological shear. CFTR activity, clearly detected in