Key Laboratory of Animal Physiology, Biochemistry and Molecular Biology of Hebei Province, College of Life Sciences, Hebei Normal University, 20 East Road of 2nd South Ring, Yuhua District, Shijiazhuang, 050024, China.
Key Laboratory of Brain Functional Genomics of Ministry of Education, School of Life Sciences, East China Normal University, Shanghai, 200062, China.
Mol Biol Rep. 2020 Dec;47(12):9811-9820. doi: 10.1007/s11033-020-06000-6. Epub 2020 Nov 23.
Idiopathic pulmonary fibrosis (IPF) is a serious life-threatening lung disease, and the median survival period of PF patients after diagnosis is only 2.5-3.5 years. At present, there are no effective drugs or therapeutics to reverse or even inhibit IPF. The main pathological characteristics of pulmonary fibrosis (PF) include damage to alveolar epithelial cells, fibroblast activation and extracellular matrix accumulation, which gradually lead to damage to the lung structure and decreased lung function. It is important to understand the cellular and molecular mechanisms of PF comprehensively and clearly. In this paper, critical signaling pathways related to PF were reviewed to present updates on the molecular mechanisms of PF.
特发性肺纤维化(IPF)是一种严重的危及生命的肺部疾病,PF 患者诊断后的中位生存期仅为 2.5-3.5 年。目前,尚无有效药物或疗法可以逆转甚至抑制 IPF。肺纤维化(PF)的主要病理特征包括肺泡上皮细胞损伤、成纤维细胞激活和细胞外基质积累,这逐渐导致肺结构损伤和肺功能下降。全面、清晰地了解 PF 的细胞和分子机制非常重要。本文综述了与 PF 相关的关键信号通路,以呈现 PF 分子机制的最新研究进展。