According to a story from EurakAlert!, a group of researchers from the University of Pennsylvania School of Medicine have achieved a major breakthrough for idiopathic pulmonary fibrosis research that will help drug developers find new treatments. For the first time, the team was able to create a mouse model of the disease using a genetic alteration that is associated with the illness.
About Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a type of progressive, chronic lung disease. It is characterized by a steady decline in lung function. The cause of this illness is not well understood, but there do appear to be some risk factors, such as smoking and exposure to certain particulate materials, such as wood, metal, stone, and coal dust, as well as silica. There is also a familial variant that has been linked to certain mutations. Symptoms of idiopathic pulmonary fibrosis include a dry cough, clubbed digits, poor exercise tolerance, shortness of breath, and distinct crackles that can be heard with a stethoscope. Treatment include supplemental oxygen, pulmonary rehabilitation, certain medications that can slow disease progression, and lung transplantation. New treatments are urgently needed, as the five year survival rate for the disease is poor at a range of 20-40 percent. To learn more about idiopathic pulmonary fibrosis, click here.
Mouse Models To The Rescue
The medical value of achieving a mouse model for idiopathic pulmonary fibrosis should not be underrated. There have been models used for testing this disease in the past, but they do not accurately reflect the mechanism of disease in idiopathic pulmonary fibrosis. This one much more closely resembles the actual conditions of the illness. Mouse models are a valuable way to observe the potential effects of a drug before clinical trials begin using human patients. It can give scientists a better understanding of what to expect before starting a trial.
Better Research, Better Treatments
The new model will allow researchers to test drugs and obtain results that will have more clinical relevance in comparison to the less accurate models that were used previously. In order to create the model, the team altered the SFTPC gene. The new model will allow scientists to observe changes in the mice over the course of disease progression. This is difficult to do with humans because idiopathic pulmonary fibrosis is often not detected until substantial progression has already occurred.