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Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease
Alveolar epithelial type 2 cell (AEC2) dysfunction is implicated in the pathogenesis of adult and pediatric interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF); however, identification of disease-initiating mechanisms has been impeded by inability to access primary AEC2s e...
Autores principales: | Alysandratos, Konstantinos-Dionysios, Russo, Scott J., Petcherski, Anton, Taddeo, Evan P., Acín-Pérez, Rebeca, Villacorta-Martin, Carlos, Jean, J.C., Mulugeta, Surafel, Rodriguez, Luis R., Blum, Benjamin C., Hekman, Ryan M., Hix, Olivia T., Minakin, Kasey, Vedaie, Marall, Kook, Seunghyi, Tilston-Lunel, Andrew M., Varelas, Xaralabos, Wambach, Jennifer A., Cole, F. Sessions, Hamvas, Aaron, Young, Lisa R., Liesa, Marc, Emili, Andrew, Guttentag, Susan H., Shirihai, Orian S., Beers, Michael F., Kotton, Darrell N. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432578/ https://www.ncbi.nlm.nih.gov/pubmed/34469722 http://dx.doi.org/10.1016/j.celrep.2021.109636 |
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