Investigation of the pathomechanisms of lung fibrosis by using bronchioalveolar epithelium-specific Pten deficient mice

特发性肺纤维化是一种慢性、致死性间质性肺疾病，由涉及上皮间质相互作用和上皮-间质转化（EMT）的异常伤口愈合引起。目的：探讨支气管肺泡上皮细胞中PTEN在肺纤维化发病机制中的生物学作用。由于异常肺纤维化引起的缺氧，KO小鼠在博来霉素给药后显示出比对照高的死亡率。KO小鼠的肺表现出肌成纤维细胞和I型胶原的积累增加。KO小鼠EMT显著增加。这些结果表明，支气管肺泡PTEN表达通过调节EMT在预防肺纤维化中起关键作用。

Idiopathic pulmonary fibrosis is a chronic, lethal interstitial lung disease resulting from abnormal wound healing that involves epithelial mesenchymal interaction and epithelial-mesenchymal transition (EMT). To clarify the biological role of PTEN, in bronchioalveolar epithelium in the pathogenesis of lung fibrosis in vivo. KO mice showed high mortality compared with the control after bleomycin administration due to hypoxia caused by aberrant lung fibrosis. Lungs of KO mice exhibited increased accumulation of myofibroblasts and type I collagen. EMT significantly increased in KO mice. These results suggested that bronchioalveolar PTEN expression plays a critical role in preventing lung fibrosis by regulating EMT.