P131 Telomere Dysfunction in Alveolar Epithelial Cells Causes Pulmonary Fibrosis: Role for TRF1

Rationale: The telomeres in type II alveolar epithelial cells (AECs) are reportedly short in patients with Idiopathic Pulmonary Fibrosis (IPF). It is unknown whether telomere shortening or telomere dysfunction directly leads to pulmonary fibrosis. The objective of this study was to investigate whether telomere dysfunction in type II AECs leads to pulmonary fibrosis in mice.Methods: To examine the consequences of type II AEC-specific telomere dysfunction, we crossed TRF1Flox/Flox (TRF1F/F) mice with mice inducibly expressing Cre recombinase …