CareLit · DOI-Nachweis · Pneumologie
Altered autophagy in the development of Hermansky-Pudlak syndrome associated Interstitial pneumonia
DOI 10.1055/s-0032-1315517
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Autor:innenS Ahuja, I Henneke, C Ruppert, L Knudsen, M Ochs, M Korfei, W Seeger, A Guenther, P Mahavadi
ZeitschriftPneumologie
Ausgabe05/2012
Jahrgang / SeitenNicht angegeben
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Abstract-Vorschau
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…
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Zitierempfehlung
S Ahuja, I Henneke, C Ruppert, L Knudsen, M Ochs, M Korfei, W Seeger, A Guenther, P Mahavadi (2012) Altered autophagy in the development of Hermansky-Pudlak syndrome associated Interstitial pneumonia. Pneumologie (05). https://doi.org/10.1055/s-0032-1315517
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