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A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling

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Woischnik, Markus ; Sparr, Christiane ; Kern, Sunčana ; Thurm, Tobias ; Hector, Andreas ; Hartl, Dominik ; Liebisch, Gerhard ; Mulugeta, Surafel ; Beers, Michael F. ; Schmitz, Gerd ; Griese, Matthias
Date of publication of this fulltext: 26 Sep 2011 09:47


We show that I73T mutation leads to impaired processing of proSP-C in alveolar type II cells, alters their stress tolerance and surfactant lipid composition, and activates cells of the immune system. In addition, we show that some of the mentioned cellular aspects behind the disease can be modulated by application of pharmaceutical drugs commonly applied in the ILD therapy.

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