LAUSR

We read with interest the article “Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype” by Declercq et al. [1]. Cystic fibrosis transmembrane conductance regulator (CFTR) is a cyclic adenosine monophosphate (cAMP)-dependent and ATP-gated Cl− channel and mutations in the CFTR gene are responsible for cystic fibrosis (CF) [2]. Declercq et al. [1] report that CFTR impairment in endothelial cells (ECs) by CFTR silencing or inhibition (using CFTR inhibitor 172, CFTRinh-172) reduced EC proliferation, migration and autophagy. Moreover, the authors demonstrated that the loss of CFTR acts in favour of EC dysfunction characterised by pro-inflammatory phenotype favouring leukocyte extravasation. Using cftr knock-out (KO) mice, Declercq et al. [1] found an increase of leukocyte extravasation in lung and liver parenchyma associated with increased levels of EC activation markers (figure 1). CFTR loss of function induces endothelial cell (EC) dysfunction. CFTR expression is reduced in pulmonary arterial ECs (PAECs) from patients with pulmonary arterial hypertension (PAH). Does CFTR loss of expression in PAECs contribute to PAH pathogenesis? https://bit.ly/3icWfa3