WISP1: A novel regulator of idiopathic pulmonary fibrosis amenable to therapeutic intervention
M Königshoff, A Jahn, K Kitowska, O Amarie, F Rose, L Fink, W Seeger, A Guenther, O Eickelberg · Pneumologie · 2008 · Heft 01
Bibliografische Angaben
Zusammenfassung
Idiopathic pulmonary fibrosis (IPF) is a devastating disease, characterized by severe loss of respiratory function due to enhanced extracellular matrix (ECM) deposition and (myo)fibroblast proliferation. Repetitive epithelial injuries with impaired alveolar wound healing represent a trigger mechanism for development of fibrosis, but molecular mechanisms linking alveolar epithelial cell (AEC) repair with development of fibrosis are poorly understood. Using whole genome microarrays, we report altered gene expression profiles of AEC derived from mice with lung fibrosis. In particular, we demonstrate that WISP1 (CCN…