P118 Reduced secretory clusterin in IPF is associated with increased elastin binding and down-regulation of fibroblast expression potentially mediated by TGF-β

Idiopathic pulmonary fibrosis (IPF) is characterized by fibroblast/myofibroblast activation and aberrant accumulation of extracellular matrix. Clusterin (CLU), a heterodimeric glycoprotein with multiple functions, including extracellular chaperoning and anti-apoptotic activity, is decreased in bronchoalveolar lavage fluid (BALF) from IPF-patients but its role in IPF is unknown. Here we investigate the …