Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis.

  title={Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis.},
  author={Andreas G{\"u}nther and Martina Korfei and Poornima Mahavadi and Daniel von der Beck and Clemens Ruppert and Philipp Markart},
  journal={European respiratory review : an official journal of the European Respiratory Society},
  volume={21 124},
Idiopathic pulmonary fibrosis (IPF) is a life-threatening condition, with a median survival of <3 yrs. The pathophysiology is not fully understood, but chronic injury of alveolar epithelial type II cells (AECII) is considered key. In IPF, disturbed folding and processing of surfactant proteins and impaired DNA repair may represent underlying reasons for maladaptive endoplasmic reticulum stress responses, increased reactive oxygen species production and/or DNA damage. Excessive AECII apoptosis… CONTINUE READING
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