Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease

著者: Konstantinos–Dionysios Alysandratos, Scott J. Russo, Anton Petcherski, Evan P. Taddeo, Rebeca Acín‐Pérez, Carlos Villacorta-Martín, Jenny Jean, Surafel Mulugeta, L. Raposo Rodríguez, Benjamin C. Blum, Ryan Hekman, Olivia T. Hix, Kasey Minakin, Marall Vedaie, Seunghyi Kook, Andrew Tilston-Lünel, Xaralabos Varelas, Jennifer Wambach, F. Sessions Cole, Aaron Hamvas, Lisa R. Young, Marc Liesa, Andrew Emili, Susan H. Guttentag, Orian S. Shirihai, Michael F. Beers, Darrell N. Kotton - Cell Reports 2021 被引用: 111

-expressing iAEC2s with hydroxychloroquine, a medication used in pediatric ILD, aggravates the observed perturbations. Thus, iAEC2s provide a patient-specific preclinical platform for modeling the epithelial-intrinsic dysfunction at ILD inception.