Inhibition of profibrotic signaling in fibroblasts from patients with IPF by the pan-histone deacetylase-inhibitor LBH589 or the IPF drug pirfenidone
M Korfei, S Skwarna, D Stelmaszek, I Henneke, BA MacKenzie, S Saito, C Ruppert, P Mahavadi, W Klepetko, S Bellusci, B Crestani, L Fink, W Seeger, OH Krämer, A Guenther · Pneumologie · 2015 · Heft 07
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 lesions called fibroblast foci (FF) is a hallmark of IPF. We investigated the therapeutic potential of pan-histone deacetylase inhibitors (HDACi), because we have discovered a significant overexpression of ClassI/II-HDAC enzymes in IPF fibroblasts/myofibroblasts. Methods: Isolated primary IPF fibroblasts were treat…
