Abstract
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.
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