Medical Faculty 'Carl Gustav Carus', Institute of Anatomy, Technical University of Dresden, Fetscherstr. 74, Dresden D-01307, Germany.
Medical Faculty 'Carl Gustav Carus', Institute of Anatomy, Technical University of Dresden, Fetscherstr. 74, Dresden D-01307, Germany
Biosci Rep. 2017 Nov 21;37(6). doi: 10.1042/BSR20171301. Print 2017 Dec 22.
Pulmonary fibrosis (PF) is characterized by inflammation and fibrosis of the interstitium and destruction of alveolar histoarchitecture ultimately leading to a fatal impairment of lung function. Different concepts describe either a dominant role of inflammatory pathways or a disturbed remodeling of resident cells of the lung parenchyma during fibrogenesis. Further, a combination of both the mechanisms has been postulated. The present review emphasizes the particular involvement of alveolar epithelial type I cells in all these processes, their contribution to innate immune/inflammatory functions and maintenance of proper alveolar barrier functions. Amongst the different inflammatory and repair events the purinergic receptor P2X7, an ATP-gated cationic channel that regulates not only apoptosis, necrosis, autophagy, and NLPR3 inflammosome activation, but also the turnover of diverse tight junction (TJ) and water channel proteins, seems to be essential for the stability of alveolar barrier integrity and for the interaction with protective factors during lung injury.
肺纤维化（PF）的特征是肺间质的炎症和纤维化，以及肺泡组织学结构的破坏，最终导致致命的肺功能损害。不同的概念描述了炎症途径的主导作用，或者在纤维化过程中肺实质常驻细胞的重塑紊乱。此外，还提出了两种机制的结合。本综述强调了肺泡上皮细胞 I 型在所有这些过程中的特殊参与，它们对固有免疫/炎症功能的贡献以及维持适当的肺泡屏障功能。在不同的炎症和修复事件中，嘌呤能受体 P2X7 是一种 ATP 门控阳离子通道，不仅调节细胞凋亡、坏死、自噬和 NLPR3 炎性小体激活，还调节多种紧密连接（TJ）和水通道蛋白的周转，对于维持肺泡屏障完整性的稳定性以及在肺损伤过程中与保护因子的相互作用似乎是必不可少的。
