Inhibition of profibrotic signaling in fibroblasts from patients with idiopathic pulmonary fibrosis by histone deacetylase-inhibitors or pirfenidone
M Korfei, S Skwarna, I Henneke, O Klymenko, G Dahlem, S Ziegler, D von der Beck, W Klepetko, L Fink, W Seeger, O Krämer, A Guenther · Pneumologie · 2014 · Heft 06
Bibliografische Angaben
Zusammenfassung
Introduction: Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by distorted pulmonary structure and the excessive deposition of extracellular matrix (ECM) proteins, such as collagen. Myofibroblasts are the primary collagen-producing cells in IPF lungs, and their accumulation within pathologic lesions called fibroblast foci (FF) is a hallmark of IPF. To explore new drugs for IPF, we investigated the therapeutic potential of histone deacetylase inhibitors (HDACi), because we have discovered a significant overexpression of Class-I/-II/-III HDAC enzymes in IPF fibroblasts/myofibroblasts. Methods:…
