JAK2 mediates lung fibrosis, pulmonary vascular remodelling and hypertension in idiopathic pulmonary fibrosis: an experimental study

• Published in: Thorax Vol. 73; no. 6; pp. 519 - 529
• Main Authors: Milara, Javier, Ballester, Beatriz, Morell, Anselm, Ortiz, José L, Escrivá, Juan, Fernández, Estrella, Perez-Vizcaino, Francisco, Cogolludo, Angel, Pastor, Enrique, Artigues, Enrique, Morcillo, Esteban, Cortijo, Julio
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.06.2018
• Subjects: Animals; Blotting, Western; Cell Proliferation - drug effects; Cells, Cultured; Clinical trials; Cytokines; Endothelial Cells; Fluorescent Antibody Technique; Gene expression; Growth factors; Humans; Hypertension, Pulmonary - drug therapy; Hypoxia; Idiopathic Pulmonary Fibrosis - drug therapy; Immunohistochemistry; Janus Kinase 2 - antagonists & inhibitors; Janus Kinase 2 - metabolism; Kinases; Medical prognosis; Myocytes, Smooth Muscle; Phenotype; Phosphorylation; Pulmonary arteries; Pulmonary fibrosis; Pulmonary hypertension; Rats; Rats, Wistar; Real-Time Polymerase Chain Reaction; RNA, Small Interfering - pharmacology; Rodents; Signal Transduction; Smooth muscle; Transforming Growth Factor beta - pharmacology; Transplants & implants; Triterpenes - pharmacology; Vascular Remodeling - drug effects; Veins & arteries; Ventilation; Spain; BKCa; idiopathic pulmonary fibrosis; JAK2; pulmonary hypertension; Pulmonary artery smooth muscle cells, Pulmonary artery endothelial cells

BackgroundPulmonary hypertension (PH) is a common disorder in patients with idiopathic pulmonary fibrosis (IPF) and portends a poor prognosis. Recent studies using vasodilators approved for PH have failed in improving IPF mainly due to ventilation (V)/perfusion (Q) mismatching and oxygen desaturatio...