Targeting the Myofibroblast in Pulmonary Fibrosis

Unbiased genomewide association studies and careful genetic studies conducted in families with high rates of pulmonary fibrosis have identified mutations in genes largely expressed in the lung epithelium (MUC5B and SFTPC) as important risk factors for the development of idiopathic pulmonary fibrosis in humans. [...]investigators have used animal models to show that deletion of telomerase (also associated with pulmonary fibrosis in humans) exclusively in the lung epithelium is sufficient to induce spontaneous pulmonary fibrosis or worsen fibrosis in response to injury (4-6). In this issue of the Journal, Bamberg and colleagues (pp. 914-927) investigated the mechanisms of apoptosis resistance in response to death receptor ligation in cultured fibroblasts and in a murine model of lung fibrosis (11).