The Role of Fibroblast Growth Factors in Idiopathic Lung Fibrosis
B MacKenzie, E El Agha, D Al Alam, K Goth, J Wilhelm, L Fink, M Königshoff, A Günther, S Bellusci · Pneumologie · 2012 · Heft 05
Bibliografische Angaben
Zusammenfassung
Idiopathic Lung Fibrosis (IPF) is a complex disease for which prognosis is poor and treatment is merely palliative. Due to the fact that patients usually present at an advanced disease state, protecting any remaining intact epithelium is imperative for preserving lung function. Fibroblast growth factors (FGFs) have been shown to have a protective effect on epithelium (FGF7), and also to maintain progenitor cell populations (FGF10). Mouse knockouts demonstrate that FGF10, and its receptor FGFR2b, are essential for lung development. In the context of lung injury, FGF10 attenuates bleomycin induced lung fibrosis du…