Enzyme Could Play Key Role in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease in which lung tissue becomes scarred over time in response to unknown injuries. This leads to shortness of breath and dry cough that gradually progresses, culminating in death in 3-5 years. The IPF lung is characterized by increases in cells in the lung called fibroblasts. We have discovered an enzyme called SHP-2 is decreased in the lung fibroblasts of patients with IPF. We will study whether reduced levels of SHP-2 from the lungs of patients with IPF render them susceptible to disease development, and whether restoring its levels in a mouse model of lung fibrosis may exert a therapeutic role.