CareLit · DOI-Nachweis · Pneumologie
Dysfunctional autophagy in Hermansky-Pudlak syndrome associated lung fibrosis
DOI 10.1055/s-0035-1556646
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Autor:innenS Ahuja, L Knudsen, S Chillappagari, I Henneke, C Ruppert, M Korfei, B Gochuico, S Bellusci, W Seeger, M Ochs, A Guenther, P Mahavadi
ZeitschriftPneumologie
Ausgabe07/2015
Jahrgang / SeitenNicht angegeben
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Abstract-Vorschau
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.…
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Zitierempfehlung
S Ahuja, L Knudsen, S Chillappagari, I Henneke, C Ruppert, M Korfei, B Gochuico, S Bellusci, W Seeger, M Ochs, A Guenther, P Mahavadi (2015) Dysfunctional autophagy in Hermansky-Pudlak syndrome associated lung fibrosis. Pneumologie (07). https://doi.org/10.1055/s-0035-1556646
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