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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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Detalhes bibliográficos
Publicado no:JCI Insight
Main Authors: 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.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2016
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Acesso em linha: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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