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Federal grant bolsters pulmonary fibrosis research

Sep. 12, 2013, 9:41 AM

Lisa Young, M.D., associate professor of Pediatrics and Medicine and Cell Biology, has been awarded a five-year, $1.9 million National Institutes of Health grant to study what causes cellular dysfunction and pulmonary fibrosis in patients with Hermansky-Pudlak syndrome (HPS).

Lisa Young, M.D.

HPS is an inherited disorder that causes albinism, decreased visual acuity, susceptibility to bleeding due to platelet dysfunction and fatal pulmonary fibrosis.

Young’s approach is to leverage the known genetic basis of this rare disease as a strategy to gain insight into critical mechanisms and pathways relevant to more common forms of pulmonary fibrosis. Currently, there are no effective treatments for pulmonary fibrosis, a condition in which there is progressive scarring in the lung leading to shortness of breath and respiratory failure.

“The specific focus of my laboratory is on the roles of alveolar epithelial cells and alveolar macrophages in the pathogenesis of pulmonary fibrosis (scarring),” she said.

Young’s laboratory plans to use mouse models to study the roles of alveolar epithelial cells and alveolar macrophages in order to understand what causes pulmonary fibrosis in HPS and how new therapies might be developed.

“I believe that this rare disease also has much to teach us about pulmonary fibrosis in general,” said Young.

Young, who is trained in both pediatric and adult pulmonary medicine, serves as director of the Rare Lung Diseases Program at the Monroe Carell Jr. Children’s Hospital at Vanderbilt, providing care for children and families with a variety of underlying interstitial lung diseases and other genetic disorders.

“My collaborators here at Vanderbilt, including Timothy Blackwell, M.D., William Lawson, M.D., and James Loyd, M.D., have a long tradition of pioneering work in the field of pulmonary fibrosis, and I am hopeful that this grant and other ongoing efforts will enable us to develop new strategies to prevent and treat these disorders.”

Young’s study, titled “Mechanism of Pulmonary Fibrosis in Hermansky-Pudlak Syndrome,” is funded by an NIH Research Project Grant (RO1) award, 1R01HL119503-01.

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