CareLit · DOI-Nachweis · Pneumologie
Deficient Autophagy in Hermansky-Pudlak Syndrome associated lung fibrosis
DOI 10.1055/s-0034-1376807
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Autor:innenS Ahuja, I Henneke, L Knudsen, BR Gochuico, C Ruppert, M Ochs, M Korfei, W Seeger, A Guenther, P Mahavadi
ZeitschriftPneumologie
Ausgabe06/2014
Jahrgang / SeitenNicht angegeben
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Abstract-Vorschau
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 type II cells (AECII) with giant lamellar bodies. We previously reported lung fibrosis in a HPSIP mouse model (HPS1/2), accompanied with surfactant accumulation and apoptosis of AE…
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Zitierempfehlung
S Ahuja, I Henneke, L Knudsen, BR Gochuico, C Ruppert, M Ochs, M Korfei, W Seeger, A Guenther, P Mahavadi (2014) Deficient Autophagy in Hermansky-Pudlak Syndrome associated lung fibrosis. Pneumologie (06). https://doi.org/10.1055/s-0034-1376807
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