Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension

Conclusions
CFTR expression is strongly decreased in pulmonary artery smooth muscle and endothelial cells in human and animal models of PH. CFTR inhibition increases vascular cell proliferation and strongly reduces pulmonary artery relaxation.

Source: European Respiratory Journal - November 25, 2021 Category: Respiratory Medicine Authors: Le Ribeuz, H., To, L., Ghigna, M.-R., Martin, C., Nagaraj, C., Dreano, E., Rucker-Martin, C., Girerd, B., Bouligand, J., Pechoux, C., Lambert, M., Boet, A., Issard, J., Mercier, O., Hoetzenecker, K., Manoury, B., Becq, F., Burgel, P.-R., Cottart, C.-H., O Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular disease Source Type: research