A Cedars-Sinai research team led by Paul W. Noble, MD, chair of the Department of Medicine and director of the Women's Guild Lung Institute, has been awarded $628,816 by California's stem cell agency to develop a treatment for idiopathic pulmonary fibrosis, a deadly disease that destroys the lungs and damages other vital organs.
The illness, which has no cure and few effective treatments, thickens and hardens lung tissue, leaving the organs badly scarred. Patients with idiopathic pulmonary fibrosis have great difficulty breathing and the chronic reduction in oxygen damages vital organs. The cause of the disease is not clearly understood and many people live only three to five years after diagnosis.
"Lung fibrosis occurs when the lung is unable to repair itself properly after injury or infection," said Noble. "Some people are more susceptible to developing fibrosis, and we currently don't understand why the normal repair and renewal of lung cells stops occurring in these patients."
The two-year study will build upon preliminary research completed at Cedars-Sinai by Noble and physician researcher Dianhua Jiang, MD, PhD. They uncovered important clues to the precise way normal lung stem cell repair occurs and how a cure might be developed.
"Currently, there is no therapy for idiopathic pulmonary fibrosis approved by the Food and Drug Administration. The only effective therapy is lung transplantation, which we do here at Cedars-Sinai," said Noble. "But if successful, our research will result in a completely novel approach to the treatment of lung diseases, allowing for the renewal and repair of the patient's cells."
This phase of Noble's research will involve the study of laboratory mice and human tissue to further identify the exact mechanisms necessary for stem cells to repair damage to the lungs.