Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs.

  title={Endoplasmic reticulum stress enhances fibrotic remodeling in the lungs.},
  author={William E. Lawson and Dong-sheng Cheng and Amber L. Degryse and Harikrishna Tanjore and Vasiliy V. Polosukhin and Xiaochuan C Xu and Dawn Catherine Newcomb and Brittany R. Jones and Juan Rold{\'a}n and Kirk B. Lane and Edward E Morrisey and Michael F. Beers and Fiona E Yull and Timothy S. Blackwell},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={108 26},
Evidence of endoplasmic reticulum (ER) stress has been found in lungs of patients with familial and sporadic idiopathic pulmonary fibrosis. We tested whether ER stress causes or exacerbates lung fibrosis by (i) conditional expression of a mutant form of surfactant protein C (L188Q SFTPC) found in familial interstitial pneumonia and (ii) intratracheal treatment with the protein misfolding agent tunicamycin. We developed transgenic mice expressing L188Q SFTPC exclusively in type II alveolar… CONTINUE READING
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