Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease
K.-D. Alysandratos, S. J. Russo, A. Petcherski, E. P. Taddeo, R. Acin-Perez, C. Villacorta-Martin, J.-C. Jean, S. Mulugeta, B. C. Blum, R. M. Hekman, M. Vedaie, S. Kook, J. A. Wambach, F. S. Cole, A. Hamvas, , D. N. Kotton
This study uses patient-derived iPSCs with an SFTPC mutation to model early alveolar epithelial dysfunction in interstitial lung disease, revealing proteostasis, metabolic, and inflammatory changes.
bioRxiv · Nov 13, 2020Cell Biology