Massachusetts General Hospital
Learning How to Stop Process in Lungs That Leads to Pulmonary Fibrosis
Pulmonary fibrosis is an often fatal condition in which the lungs fill with scar tissue. The process is thought to be triggered by damage to the epithelial cells that line the lung's air sacs. The damage activates cells called fibroblasts that then make scar tissue. To learn how to stop this process, we will first use human cells to study the interaction of epithelial cells and fibroblasts. We will also use genetically modified mice that have a label in the epithelial cells of the lung, allowing us to specifically examine these cells. Using a model of pulmonary fibrosis, we will determine what new proteins are made by epithelial cells that activate fibroblasts. This project should reveal new therapeutic strategies to combat fibrosis.
Update: Using optimized cell culture techniques and mice with specific genetic labels, we are better defining the stress responses of alveolar (air sac) epithelial cells to lung injury. We are also determining the mechanisms by which injury to these cells leads to their release of fibroblast-activating substances. In so doing, we are aiming hoping to identify new targets for drug therapies to halt, or better yet, to reverse the relentless progression of this disease.