Article
Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease.
Cell reports - 31 Aug 2021
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
Abstract excerpt
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 early on. Here, we present a human in vitro model permitting investigation of epithelial-intrinsic events culminating...
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