CareLit · DOI-Nachweis · Pneumologie
Role of impaired lysosomal trafficking in a murine model of Hermansky-Pudlak-syndrome associated interstitial pneumonia (HPSIP)
DOI 10.1055/s-0029-1202428
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Nachweis in CareLit
Autor:innenP Mahavadi, M Korfei, C Ruppert, I Henneke, G Liebisch, G Schmitz, G Bernadette, W Seeger, A Günther
ZeitschriftPneumologie
Ausgabe02/2009
Jahrgang / SeitenNicht angegeben
Kernaussage aus der Literatur
Abstract-Vorschau
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 hu…
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Zitierempfehlung
P Mahavadi, M Korfei, C Ruppert, I Henneke, G Liebisch, G Schmitz, G Bernadette, W Seeger, A Günther (2009) Role of impaired lysosomal trafficking in a murine model of Hermansky-Pudlak-syndrome associated interstitial pneumonia (HPSIP). Pneumologie (02). https://doi.org/10.1055/s-0029-1202428
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