Biological role of the proapoptotic transcription factor CHOP in Idiopathic Pulmonary Fibrosis (IPF)

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive diffuse parenchymal lung disease (DPLD), with a median survival time of less than three years following diagnosis. The precise mechanism of IPF is still not fully understood but chronic injury of alveolar epithelial type-II cells (AECII) is increasingly accepted as key event. We have recently identified severe Endoplasmic Reticulum (ER) stress leading to apoptosis as a major pathomechanism for such alveolar epithelial cell injury. In line with this, the pro-apoptotic transcription factor CHOP, which functions as a crucial mediator of ER…