Altered autophagy in the development of Hermansky-Pudlak syndrome associated Interstitial pneumonia

Introduction: Hermansky-Pudlak syndrome (HPS) is a lysosome realated disorder. Patients with HPS types -1, -2 & -4 develop pulmonary fibrosis called Hermansky-Pudlak syndrome associated Interstitial Pneumonia (HPSIP). HPSIP lungs show enlarged alveolar typeII cells (AECII) with giant lamellar bodies. We previously reported lung fibrosis in a HPSIP mouse model (HPS1/2), defective surfactant accumulation and apoptosis of AECII due to severe lysosomal stress and ER stress in these mice as well as in human HPS1. Here, we aim to analyze autophagy, an important lysosomal degradation pathway, under HPSIP conditions. Me…