Abstract：
Idiopathic pulmonary fibrosis is a progressive lung disease of unknown etiology characterized by distorted distal lung architecture, inflammation, and fibrosis. Several lung cell types, including alveolar epithelial cells and fibroblasts, have been implicated in the development and progression of fibrosis. However, the pathogenesis of idiopathic pulmonary fibrosis is still incompletely understood. The latest research has found that dysregulation of lipid metabolism plays an important role in idiopathic pulmonary fibrosis. The changes in the synthesis and activity of fatty acids, cholesterol and other lipids seriously affect the regenerative function of alveolar epithelial cells and promote the transformation of fibroblasts into myofibroblasts. Mitochondrial function is the key to regulating the metabolic needs of a variety of cells, including alveolar epithelial cells. Sirtuins located in mitochondria are essential to maintain mitochondrial function and cellular metabolic homeostasis. Sirtuins can maintain normal lipid metabolism by regulating respiratory enzyme activity, resisting oxidative stress, and protecting mitochondrial function. In this review, we aimed to discuss the difference between normal and idiopathic pulmonary fibrosis lungs in terms of lipid metabolism. Additionally, we highlight recent breakthroughs on the effect of abnormal lipid metabolism on idiopathic pulmonary fibrosis, including the effects of sirtuins. Idiopathic pulmonary fibrosis has its high mortality and limited therapeutic options; therefore, we believe that this review will help to develop a new therapeutic direction from the aspect of lipid metabolism in idiopathic pulmonary fibrosis.
摘要：
特发性肺纤维化是一种病因不明的进行性肺病，其特征是远端肺结构扭曲，炎症,和纤维化。几种肺细胞类型，包括肺泡上皮细胞和成纤维细胞，与纤维化的发展和进展有关。然而,特发性肺纤维化的发病机制尚不完全清楚。最新研究发现,脂质代谢紊乱在特发性肺纤维化中起重要作用。脂肪酸合成和活性的变化，胆固醇等脂类严重影响肺泡上皮细胞的再生功能,促进成纤维细胞向肌成纤维细胞转化。线粒体功能是调节多种细胞代谢需求的关键,包括肺泡上皮细胞。位于线粒体中的Sirtuins对于维持线粒体功能和细胞代谢稳态至关重要。Sirtuins可以通过调节呼吸酶活性来维持正常的脂质代谢，抗氧化应激，保护线粒体功能.在这次审查中,我们旨在讨论正常和特发性肺纤维化肺在脂质代谢方面的差异。此外,我们强调最近关于脂质代谢异常对特发性肺纤维化的影响的突破，包括沉默调节蛋白的作用。特发性肺纤维化具有高死亡率和有限的治疗选择;因此,我们认为，这篇综述将有助于从脂质代谢方面开拓特发性肺纤维化的新治疗方向。
