Role of impaired lysosomal trafficking in a murine model of Hermansky-Pudlak-syndrome associated interstitial pneumonia (HPSIP)
P Mahavadi, M Korfei, C Ruppert, I Henneke, G Liebisch, G Schmitz, G Bernadette, W Seeger, A Günther · Pneumologie · 2009 · Heft 02
Bibliografische Angaben
Zusammenfassung
Rationale: Hermansky-Pudlak Syndrome (HPS) is characterized by a defective lysosomal transport and an abnormal morphological appearance of alveolar type II cell (AECII) lamellar bodies. Some forms of HPS are associated with development of lung fibrosis. In the present study, we investigated the role of apoptosis in HPS1/2 (ep/pe) and HPS1/6 (ep/ru) double mutant mice, their corresponding single mutant mice and in human HPS1 patients. Methods: Lung tissue and bronchoalveolar lavage fluids (BALF) from the above mentioned HPS mice were analyzed by western blotting for SP-B, SP-C, lysosomal aspartyl protease (Cathep…
