The American Thoracic Society Foundation has teamed with the American Lung Association to co-fund a two-year $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.
HPS is known to cause fibrosis, or scarring, of the lungs, and gaining a more detailed understanding of the disease may accelerate the discovery of therapies for more common lung diseases, such as cystic fibrosis and idiopathic pulmonary fibrosis.
The 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, Boston.
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.”