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...

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Detalles Bibliográficos
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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2021
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