Areas of Interest
The alveolar epithelium plays a critical role in maintaining normal lung function. Type I Alveolar Epithelial Cells (AEC1s) provide broad coverage of the alveolar surface and serve as the interface for gas exchange. Type II Alveolar Epithelial Cells (AEC2s) play multiple essential roles including the production of pulmonary surfactant and serving as progenitor cells capable of differentiating into AEC1s during homeostasis and after lung injury. Defects in AEC1 regeneration underlie severe pulmonary diseases such as idiopathic pulmonary fibrosis (IPF), a progressive scarring disease that results in gradual deterioration of lung function leading to respiratory failure and death within 3-5 years. Our laboratory and others have demonstrated that the specific defect in epithelial regeneration that drives the pathogenesis of fibrosis is impaired AEC2 to AEC1 differentiation.