Idiopathic Pulmonary Fibrosis

Our main focus of research is Idiopathic Pulmonary Fibrosis and the fibroblast.
Idiopathic pulmonary fibrosis (IPF) is a devastating and fatal interstitial lung disease which affects over 5 million people worldwide. IPF mostly affects people from the age of 55, however it can affect people younger than 50. The average survival time ranges from 3 to 5 years post diagnosis. There is no cure for IPF and few therapeutic options apart from a lung transplant. The current theory for the mechanism of IPF is that it is a disease driven by unregulated wound repair. Wound repair is mostly driven by fibroblast cells. These cells in a normal environment are dormant, however in the presence of an injury are activated to initiate wound repair. This process includes proliferation of fibroblasts themselves and production of extracellular matrix (ECM) which serves as a scaffold for wound repair. Under normal circumstances once the repair is complete, this scaffold is degraded and the fibroblasts retreated. However in the IPF lung it is believe that the fibroblasts become disconnected from the end stage signals of wound repair. As a result they continue to replicate and product ECM which distorts the lung, interfering with the normal function of gaseous exchange.
In our lab, using cell biology and molecular techniques, we are focused on fibroblast biology. We are working on why the fibroblast in IPF is disconnected from its normal signaling mechanisms, why it continues to replicate in the absence of a wound and why it continues to deposit ECM to the detriment of the lung.