Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease
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
Abstract
-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.
Topics & Concepts
Induced pluripotent stem cellReprogrammingInterstitial lung diseaseBiologyLungProgenitor cellCell biologyImmunologyPathologyMedicineCancer researchStem cellCellGeneGeneticsInternal medicineEmbryonic stem cellNeonatal Respiratory Health ResearchInterstitial Lung Diseases and Idiopathic Pulmonary FibrosisMedical Imaging and Pathology Studies