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Survival Improvement in Pulmonary Fibrosis Possible with the Aid of Protein Molecule

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Researchers from the University of Illinois at Chicago College of Medicine have recently discovered a protein molecule that appears to slow the development of pulmonary fibrosis, a progressive lung disease that is often fatal three to five years following prognosis.

Their finding was reported in the American Journal of Respiratory and Critical Care Medicine.

Almost five million people are affected by pulmonary fibrosis around the world, which causes the lungs to become littered in fibrous scar tissue and can result in shortness of breath that becomes more and more serious as the disease develops.

Chronic inflammatory and autoimmune diseases can cause pulmonary fibrosis, as well as exposure to asbestos, certain toxic gases, and even radiation therapy to treat lung cancer.

Treatment options are limited due to the scarring that occurs, remains permanent. As of today lung transplantation remains the only effective method of treatment, but it is usually reserved for advanced cases.

"Finding a new therapeutic target for the treatment of pulmonary fibrosis is exciting, especially because the therapies available generally only slow the disease in very few patients," said UIC postdoctoral research associate in pharmacology and first author of the paper, Long Shuang Huang.lung protein

In a prior genetic studies of patients with idiopathic pulmonary fibrosis, where no cause can be distinguished, the researchers discovered differences in some genes known to be involved in pulmonary fibrosis, including in the gene coding for a protein called lysocardiolipin acyltransferase, or LYCAT.

In order to inspect and explore the potential role of LYCAT in pulmonary fibrosis, the researchers measured its levels in the blood of idiopathic pulmonary fibrosis patients. Patients with the highest levels of LYCAT had considerably better lung function and higher three-year survival rates than those with lower levels.

"Since higher LYCAT levels directly correlate with better lung function and outcomes, we think the protein is playing some kind of protective role, or could be slowing the progress of pulmonary fibrosis. This suggests that boosting LYCAT levels in patients with pulmonary fibrosis may be a viable new therapeutic approach to treating the disease," Huang said.

Furthermore, the researchers also studied the role of LYCAT in a mouse model of lung tissue scarring, and discovered that in mice where the LYCAT gene was a total non-factor, scar tissue progressed more rapidly as opposed to the mice that had the gene. In mice engineered to generate elevated levels of LYCAT the overall progression of scarring was significantly slower.

Looking for compounds or small molecules that increase the production of LYCAT is the next phase of the research for Huang and his peers.
