Endothelial CFTR dysfunction and its involvement in the pathogenesis of pulmonary arterial hypertension

F. Antigny and co-workers raise interesting points in their comment on the transcriptomic analysis of cystic fibrosis transmembrane conductance regulator (CFTR)-impaired endothelial cells (ECs) revealing a pro-inflammatory phenotype [1]. Indeed, CFTR impairment different models organisms, absence concomitant infection, suggests that ECs are an overlooked mediator exaggerated observed (CF). Whether is involved other vascular diseases intriguing question. recent report indicates pivotal role pulmonary arterial hypertension (PAH) [2]. The dysfunction goes beyond by playing