Idiopathic pulmonary fibrosis (IPF) is an agnogenic chronic interstitial pneumonia, characterized by interstitial fibrosis, differentiation of myofibroblasts, deposition of extracellular matrix, finally leads to distortion of lung architecture and insufficiency of respiratory.Once diagnosed IPF, the treatment options are limited.The unknown microdamage of alveolar has been regarded as the initial factor of IPF, consequently leads to the activation of transforming growth factor-β and the destruction of alveolar basement membrane.The activated transforming growth factor-β can induce the apoptosis of epithelium cells, epithelial-mesenchymal transition, differentiation from fibroblasts to myofibroblasts, and deposition of extracellular matrix, eventually loss of respiratory function. Key words: Idiopathic pulmonary fibrosis; Transforming growth factor-β; Alveolar epithelial cell; Pulmonary interstitium
Nasreen KhalilRobert O’ConnorHelmut UnruhPeter WarrenAngela KempHarry B. Greenberg
Deborah L. W. ChongTheresia MikolaschJagdeep SahotaCarine RebeyrolHelen GarthwaiteHelen BoothMelissa HeightmanEmma DennenyRicardo J. JoséAkif A. KhawajaAnna DuckworthMyriam LabelleChris J. ScottonJoanna C. Porter
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