Alveolar Injury and Regeneration Following Deletion of ABCA3

Surfactant deficiency, diffuse alveolar damage and respiratory failure caused by loss of Abca3 in AT2 cells was followed by remarkable proliferation of alveolar cells and selective survival of ABCA3 sufficient cells resulting in regeneration of alveolar structure and function, providing the conceptual framework for the development of therapies to ameliorate lung diseases caused by mutations in ABCA3 and other genes critical for AT2 cell function or surfactant homeostasis. Overall design: Control and Abca3 cKO AT2 cell RNA-seq at 6 days post tamoxifen in adult mice.

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Rindler TN, Stockman CA, Filuta AL, Brown KM, Snowball JM, Zhou W, Veldhuizen R, Zink EM, Dautel SE, Clair G, Ansong C, Xu Y, Bridges JP, Whitsett JA