Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.

Hélène Le RibeuzLucie ToMaria-Rosa GhignaClémance MartinChandran NagarajElise DreanoCatherine Rucker-MartinBarbara GirerdJérôme BouligandChristine PechouxMélanie LambertAngèle BoetJustin IssardOlaf MercierKonrad HoetzeneckerBoris ManouryFrédéric BecqPierre Regis Burgel Charles-Henry Cottart Andrea J OlschewskiIsabelle Sermet-GaudelusFrédéric PerrosMarc Humbert I David Montani Fabrice Antigny

Published in: The European respiratory journal (2021)

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.

Keyphrases

pulmonary artery
pulmonary arterial hypertension
cystic fibrosis
endothelial cells
smooth muscle
pulmonary hypertension
cell proliferation
coronary artery
poor prognosis
high glucose
cell cycle
vascular endothelial growth factor
induced pluripotent stem cells
binding protein
pi k akt
pluripotent stem cells