Characterization of the antifibrotic efficacy of PBI-4050 in AdTGF-?1 mediated pulmonary fibrosis
Fibroproliferative disorders are a/the leading cause of morbidity and mortality worldwide. A large group of interstitial lung diseases (ILD) can cause progressive scarring of lung tissues, affecting the ability to breathe. Idiopathic Pulmonary Fibrosis (IPF) is one of the major forms of ILD and is characterized by a progressive decline in lung function. Although the pathogenesis of IPF is incompletely understood, work from our group and others suggest that transforming growth factor beta 1 (TGF-?1) is involved in the fibrogenic process. PBI-4050 is a promising novel anti-fibrotic drug developed by ProMetic Life Sciences that is currently in phase II clinical trial in IPF. In this project, we will examine the anti-fibrotic effect of PBI-4050 in a TGF-?1 mediated animal model of lung fibrosis. This work will increase our understanding of the cellular and molecular mechanisms involved in pulmonary fibrosis and potentially lead to the development of new therapeutic strategies for IPF.