Brigham and Women's Hospital
Identifying the Mechanisms Underlying Hermansky-Pudlak Syndrome
Hermansky-Pudlak syndrome (HPS) is a rare inherited disease which causes scarring of the lungs with limited treatment options. We 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 identifying new treatment targets. Understanding the underlying mechanisms of HPS may accelerate the discovery of therapies for more common lung diseases like cystic fibrosis and idiopathic pulmonary fibrosis.