MAP1LC3B overexpression protects against Hermansky-Pudlak syndrome type-1-induced defective autophagy in vitro

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder, and some patients with HPS develop pulmonary fibrosis, known as HPS-associated interstitial pneumonia (HPSIP). We have previously reported that HPSIP is associated with severe surfactant accumulation, lysosomal stress, and alveo...

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Publicat a:Am J Physiol Lung Cell Mol Physiol
Autors principals: Ahuja, Saket, Knudsen, Lars, Chillappagari, Shashi, Henneke, Ingrid, Ruppert, Clemens, Korfei, Martina, Gochuico, Bernadette R., Bellusci, Saverio, Seeger, Werner, Ochs, Matthias, Guenther, Andreas, Mahavadi, Poornima
Format: Artigo
Idioma:Inglês
Publicat: American Physiological Society 2015
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4888554/
https://ncbi.nlm.nih.gov/pubmed/26719147
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1152/ajplung.00213.2015
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