Year 1

Chronic lung disease is an enormous societal and medical problem in California and the nation as a whole, representing the third most likely cause of death with treatment costs of $389.2 billion in 2011 and expected to reach $832.9 billion in 2021 according to the Milken Institute. Chronic lung diseases cover a spectrum of disorders that include pulmonary fibrosis, a disease that makes it difficult to breathe due to the accumulation of scar tissue in the lung, and chronic obstructive pulmonary disease (COPD), a disease that makes breathing difficult due to loss of critical structures that allow oxygen to enter the blood. According to Breathe California, COPD is the 4th leading cause of death in the United States and 1.6 million Californians are diagnosed with COPD. Treatment options vary by disease but are particularly ineffective for patients with COPD and fibrotic lung diseases. One fibrotic lung disease termed idiopathic pulmonary fibrosis (IPF) can only be treated by lung transplantation and this option is limited to those who meet specific age and health criteria. Without transplantation the majority of IPF patients die within three years of initial diagnosis. Our research program has relocated from Duke University Medical Center to Cedars-Sinai Medical Center with the goal of translating a basic understanding of disease mechanisms to improved patient care. We have developed new tools to understand cell types and regulatory mechanisms that participate in lung repair and disease in mouse models. These new mouse models allow us to investigate ways that lung cells interact to repair damage in an intact animal model, and have allowed us to develop new ways of reconstructing regulatory cell interactions in a culture environment. Parallel culture models for analysis of human lung cell types have allowed us to investigate the critical roles for cell-cell interactions in the regulation of epithelial repair and aberrant tissue remodeling. Our long-term goal is to determine how lung disease is caused and use this information to develop new therapies that will prevent either initiation or progression of lung disease.