The words pulmonary fibrosis literally translates to lung scarring; the condition encompasses an abnormal formation of scar tissue in the lungs. The scar tissue builds up in the walls of the air sacs known as alveoli, and eventually makes it hard for air to pass through. Pulmonary fibrosis can be mild, severe or often life-threatening.
The cause of IPF is unknown but it is widely accepted that repeated injury to the epithelium leads to dysregulated healing, initiating a cascade of processes resulting in fibroblast / myofibroblast accumulation and overproduction and deposition of collagen. The Tissue Repair Unit continues to pioneer studies in identifying the gp130-induced STAT3 signalling the pathway as pivotal in the development of lung fibrosis. What regulates STAT3-mediate fibrosis is not clear, but current studies are focusing on understanding the role of mediators known to activate the pathway, cell types that may be regulating the mediator response, as well as a possible breakdown in regulation of the naturally occurring inhibitors that normally control the STAT3 response.
CURRENT RESEARCH ACTIVITIES
The Tissue Repair Group have an extensive programme of research which investigates the cellular and molecular pathways driving IPF. Notably, they have received NHMRC project grant funding for the three project listed below.
- STAT3 regulation of cell responses in IPF
- Epithelial-mesenchymal cell communication towards new therapeutic targets for fibrosis
- Fibroblast Scenecence as a driver of pulmonary fibrosis
- The Immune regulation of Idiopathic Pulmonary Fibrosis
Through a local, national and international collaboration, the Unit continues to investigate cross talk between epithelial cells and fibroblasts and the role this plays in the progression
and development of fibrosis.
The Tissue Repair Group continue to dissect the molecular mechanisms and cell signalling pathways driving fibrosis, and together with collaborations at the University of Newcastle, are investigating mitochondrial dysfunction in IPF.