Dysfunctional autophagy in Hermansky-Pudlak syndrome associated lung fibrosis
S Ahuja, L Knudsen, S Chillappagari, I Henneke, C Ruppert, M Korfei, B Gochuico, S Bellusci, W Seeger, M Ochs, A Guenther, P Mahavadi · Pneumologie · 2015 · Heft 07
Bibliografische Angaben
Zusammenfassung
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder and HPS types 1, 2 and 4develop pulmonary fibrosis referred to as Hermansky-Pudlak syndrome associated interstitial pneumonia (HPSIP). We have previously reported spontaneous lung fibrosis in HPS1/2 double mutant mice primarily associated with surfactant accumulation, severe lysosomal stress and alveolar epithelial cell type II (AECII) apoptosis. In the current study we hypothesized that autophagy, a lysosome mediated degradation pathway might be altered, resulting in the excessive lysosomal stress that is reported under HPSIP conditions. Hen…
