New research is helping experts understand the underlying causes of idiopathic pulmonary fibrosis on the molecular scale. When solving any problem, disease included, it is best to start by understanding the underlying cause. Now researchers say that the cause of pulmonary fibrosis is a reduction in activity in the transcription factor FoxO3, or at the very least that it plays a significant role in the progression of the disease.
Idiopathic pulmonary fibrosis is a chronic disease in which the ability of the lung to function begins to progressively worsen. The process in which the lungs transfer oxygen to the blood becomes less efficient, and the disease causes severe scarring in the lungs. Symptoms include shortness of breath, a dry cough, and clubbed digits. Smoking is a significant risk factor. Men are more often affected than women. The five year survival rate for the condition is only forty percent at best. A double lung transplant is often the best way to improve survival, but is no guarantee. You can read more about this disease here.
Connective tissue cells, or fibroblasts, undergo changes in the disease. Normally, they provide structure for the air sacs in the lungs. In the disease, their composition changes and they contain more contractile proteins. This process is what causes the scarring and changes in lung tissue. Blood vessels are also damaged, causing the characteristic short breath.
These changes are linked to the reduction in FoxO3 factor activity. The team developed a mouse model of the disease to test their findings. Using mice that were modified to have less FoxO3, the researchers then used an experimental treatment called UCN-01, which activates FoxO3. The treatment is currently being tested as a tumor treatment.
The use of UCN-01 reduced symptoms and served to improve lung function in the mice during the experiment.
