Deficient Autophagy in Hermansky-Pudlak Syndrome associated lung fibrosis
S Ahuja, I Henneke, L Knudsen, BR Gochuico, C Ruppert, M Ochs, M Korfei, W Seeger, A Guenther, P Mahavadi · Pneumologie · 2014 · Heft 06
Bibliografische Angaben
Zusammenfassung
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 type II cells (AECII) with giant lamellar bodies. We previously reported lung fibrosis in a HPSIP mouse model (HPS1/2), accompanied with surfactant accumulation and apoptosis of AECII due to severe lysosomal stress and ER stress. Data from human HPS1 patient corroborated with the HPS1/2 mice data. Here, we aim to analyze autophagy, an important lysosomal degradation pathway, un…
