Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disease where scar tissue accumulates in the lungs and decreases the air space required to breath.
This process known as fibrosis results in reduced breathing efficiency and ultimately death. The lungs undergo structural changes making them stiff and less flexible and consequently inflating and deflating your lungs becomes ever more difficult.
The condition affects males more than females and survival rates are worse than most cancers.
IPF cases are increasing year upon year but there is currently no cure available. This highlights the importance of research in this area and a real need to understand the mechanism driving IPF to generate effective medicines.
Dr Amanda Tatler has previously shown that IPF patients seem to have a significantly lower amount of a protein called Elk-1 in their lungs.
Leading on from this, my area of research looks at the function of this protein in this disease and the impact of its absence.
Our aims are to firstly, find out whether the absence of this protein alone is enough to trigger the fibrotic process and secondly, investigate why IPF patients lack this particular protein. We are particularly interested in whether a deficiency in Elk1 causes age-related changes in the lungs that could progress to the development of fibrosis.
Our results show that when Elk1 protein is lost, small patches of scar tissue begin to form, and the lung structure starts to change and resemble early stages of fibrosis. We have developed our investigations on these initial findings and have also addressed the effect that loss of Elk1 has on scarring in other organs besides the lungs.
Watch this space for the publication.