@Article {Kim2000652，作者= {Kim，Sang-Hun和Lee，Jung Yeon和Yoon，Chang Min和Shin和Shin，Hyeon Jun和Lee，Sei Won以及Rosas，Ivan和Herzog，Erica和Dela Cruz，Charles和Charles and Charles and Kaminski，Naftalii和Kang，min-jong}，title = {线粒体抗病毒信号传导蛋白对于肺纤维化的发展至关重要= {188bet官网地址欧洲呼吸社会}，摘要= {危险信号或与损伤相关的分子模式（潮湿），探讨线粒体先天免疫反应，其中线粒体抗病毒信号蛋白（MAVS）是一个关键平台，可以作为关键平台分子进行介导它们。然而，尚未鉴定出MAV在特发性肺纤维化（IPF）的发病机理中的作用。也没有探索当前现有药物可以调节MAVS信号传导的可能性。在这里，使用已建立的肺纤维化模型，我们证明了MAV作为多个潮湿信号通路的关键介质，以及在体内造成的霉素诱导损伤后随之而来的肺纤维化。博来霉素损伤后，主要在巨噬细胞中观察到MAV的表达。此外，多聚体MAVS聚集是MAVS信号激活的关键事件，在博来霉素受伤的肺中显着增加并持续存在。有趣的是，促凋亡的BH3模拟ABT-263减弱了MAV的表达及其信号传导，因此，实验性肺纤维化的发展。相比之下，两种经批准的IPF治疗药物的Pirfenidone或Nintedanib的治疗作用与MAV或其信号传导的调节无关。 Importantly, multimeric MAVS aggregation was significantly increased in lungs from the patients with IPF as well. In conclusion, MAVS may play an important role in the development of pulmonary fibrosis, and targeting MAVS with BH3 mimetics may provide a novel therapeutic strategy for IPF, a major unmet disorder.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Yan reports grants from National Institutes of Health, during the conduct of the study;.Conflict of interest: Dr. Kim has nothing to disclose.Conflict of interest: Dr. Lee has nothing to disclose.Conflict of interest: Dr. Yoon has nothing to disclose.Conflict of interest: Dr. SHIN has nothing to disclose.Conflict of interest: Dr. Lee has nothing to disclose.Conflict of interest: Dr. Rosas has nothing to disclose.Conflict of interest: Dr. Herzog reports personal fees from Boehringer, personal fees from MErck, personal fees from Genentech, grants from Boehringer, grants from Sanofi, grants from Bristol Myers, outside the submitted work;.Conflict of interest: Dr. Cruz has nothing to disclose.Conflict of interest: Dr. Kaminski reports personal fees from Biogen Idec, Boehringer Ingelheim, Third Rock, Miragen, Pliant, personal fees from Samumed, NuMedii, Indaloo, Theravance, LifeMax, Three Lake Partners, outside the submitted work; In addition, Dr. Kaminski has a patent New Threapies in Pulmonary Fibrosis with royalties paid to Biotech, and a patent Peripheral Blood Gene Expression issued and Serves as Deputy Editor of Thorax, BMJ. .Dr. Kaminski reports personal fees from Biogen Idec, personal fees from Boehringer Ingelheim, personal fees from Third Rock, personal fees from MMI, non-financial support from Miragen, personal fees from Pliant, personal fees from Samumed, personal fees from NuMedii, personal fees from Indaloo, outside the submitted work; In addition, Dr. Kaminski has a patent New Threapies in Pulmonary Fibrosis with royalties paid to Biotech, and a patent Peripheral Blood Gene Expression issued and Serves as Deputy Editor of Thorax, BMJ.Conflict of interest: Dr. Kang has nothing to disclose.}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/early/2020/10/15/13993003.00652-2020}, eprint = {//www.qdcxjkg.com/content/early/2020/10/15/13993003.00652-2020.full.pdf}, journal = {European Respiratory Journal} }