Mechanisms underlying the pathogenesis of pulmonary fibrosis remain incompletely understood. Emerging evidence suggests changes in mitochondrial quality control are a critical determinant in many lung diseases, including chronic obstructive pulmonary disease, asthma, pulmonary hypertension, acute lung injury, lung cancer, and in the susceptibility to pulmonary fibrosis. Once thought of as the kidney-bean shaped powerhouses of the cell, mitochondria are now known to form interconnected networks that rapidly and continuously change their size to meet cellular metabolic demands. Mitochondrial quality control modulates cell fate and homeostasis, and diminished mitochondrial quality control results in mitochondrial dysfunction, increased reactive oxygen species (ROS) production, reduced ATP production, and often induces intrinsic apoptosis. Here, we review the role of the mitochondria in alveolar epithelial cells, lung macrophages, and fibroblasts within the context of pulmonary fibrosis.

肺纤维化发病机理的机制尚不完全清楚。越来越多的证据表明，线粒体质量控制的变化是许多肺部疾病的关键决定因素，包括慢性阻塞性肺疾病，哮喘，肺动脉高压，急性肺损伤，肺癌以及肺纤维化的易感性。线粒体曾经被认为是细胞的豆角形动力源，现在可以形成相互连接的网络，这些网络可以快速，连续地改变其大小，以满足细胞的新陈代谢需求。线粒体质量控制调节细胞命运和体内稳态，而线粒体质量控制的减少导致线粒体功能障碍，活性氧（ROS）产生增加，ATP产生减少，并经常诱发内在凋亡。这里，