The American Thoracic Society Foundation and the American Lung Association announced today that they are co-funding an $80,000 grant that will support important research into the mechanisms underlying Hermansky-Pudlak syndrome (HPS), a rare inherited disease which affects a number of organs including the lungs.
Understanding HPS, which causes fibrosis, or scarring, of the lungs, may accelerate the discovery of therapies for more common lung diseases like cystic fibrosis and idiopathic pulmonary fibrosis.
The two-year, $80,000 ATS Foundation/American Lung Association Research Grant will fund research being performed by Souheil El-Chemaly, MD, MPH, assistant professor in the Division of Pulmonary and Critical Care Medicine and the clinical director of the Center for LAM Research and Clinical Care at Brigham and Women's Hospital.
Dr. El-Chemaly's study, "Zebrafish xenotransplantation model to study HPS pulmonary fibrosis," will focus on cells called fibroblasts, which are involved in lung fibrosis in HPS patients. His team will explore how HPS fibroblasts differ from those in normal lungs and whether treatment can make them behave like normal fibroblasts. These studies will be performed in zebrafish embryos, a new model for studying HPS pulmonary fibrosis which may have use in subsequent studies assessing the effectiveness of new treatments. The researchers will also explore whether measurement of proteins derived from HPS fibroblasts in the blood of HPS patients can predict disease severity.
"Our research will not only help identify new treatment targets for this devastating disease but our new disease model will help in future research efforts," says Dr. El-Chemaly. "Our findings will also have implications for studies of treatments for pulmonary fibrosis not caused by HPS. I am honored that the ATS and the American Lung Association have recognized the value of our work."
Renewing a relationship between the ATS and the Lung Association that dates back over a hundred years, the ATS Foundation and the Lung Association plan to co-fund a grant for lung cancer research next year and look forward to supporting research into other respiratory diseases in the future.
Current levels of funding for respiratory research from government and other sources do not reflect the enormous medical, economic, and societal burden of respiratory disease in the United States."If we are to advance the speed of discovery to find better ways to prevent and cure lung disease," according to Stephen Crane, PhD. ATS Executive Director, "even greater collaborative effort between the ATS and the Lung Association will be required in the future. ATS commits to this goal."
The funding provided by the ATS Foundation/American Lung Association Research grants will help close this gap and further the missions of both organizations to improve the respiratory health of patients worldwide. "We welcome this chance to combine resources with the American Thoracic Society and support this promising research that might not have been possible without this collaboration," said Harold Wimmer, National President and CEO of the American Lung Association. "An exciting aspect of this research is that it may impact several types of deadly lung diseases. We look forward to more opportunities like this to partner with ATS in the future."