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Epithelial-macrophage interactions determine pulmonary fibrosis susceptibility in Hermansky-Pudlak syndrome

Alveolar epithelial cell (AEC) dysfunction underlies the pathogenesis of pulmonary fibrosis in Hermansky-Pudlak syndrome (HPS) and other genetic syndromes associated with interstitial lung disease; however, mechanisms linking AEC dysfunction and fibrotic remodeling are incompletely understood. Since...

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Publicat a:JCI Insight
Autors principals: Young, Lisa R., Gulleman, Peter M., Short, Chelsi W., Tanjore, Harikrishna, Sherrill, Taylor, Qi, Aidong, McBride, Andrew P., Zaynagetdinov, Rinat, Benjamin, John T., Lawson, William E., Novitskiy, Sergey V., Blackwell, Timothy S.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Clinical Investigation 2016
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC5070955/
https://ncbi.nlm.nih.gov/pubmed/27777976
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.88947
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