PT - 杂志Au - Kim，Sang-Hun Au - Lee，Jung Yeon Au - Yoon，Chang Min Au - Shin，Hyeon Jun Au - Lee，Sei Won Au - Rosas，Ivan Au - Herzog，Erica Au - Dela Cruz，Charles Au - Kaminski，Naftali Au - Kang，Min-Jong Ti - 线粒体抗病毒信号蛋白是肺纤维化辅助辅助助剂的发展至关重要 - 10.1183 / 13993003.00652-2020 DP - 2020年1月01日 - 欧洲呼吸期刊PG - 2000652 4099 - HTTP：//www.qdcxjkg.com/content/early/2020/10/15/13993003.00652-2020.short 4100 - //www.qdcxjkg.com/content/early/2020/10/15/13993003.00652-2020全AB - 危险信号或损伤相关的分子模式（潮湿），施加线粒体先天性免疫应答，其中线粒体抗病毒信号传导蛋白（MAVS）作为介导它们的关键平台分子。然而，MAVS在发作性肺纤维化（IPF）发病机制中的作用尚未识别。也可以通过目前现有药物调制MAVS信号传导的可能性。在这里，使用型肺纤维化模型，我们证明了MAVS作为多次潮湿信号通路的临界介体和岩土霉素诱导的体内损伤后的随后的肺纤维化。在博来霉素损伤后，主要在巨噬细胞中观察到MAVS的表达。此外，多聚体MAVS聚集，MAVS信号激活的关键事件，在博莱霉素受伤的肺部显着增加，持续存在。有趣的是，Proapoftotic BH3模仿ABT-263衰减了MAVS及其信号传导的表达，并因此探测了实验性肺纤维化的发展。相比之下，Pirfenidone或尼林尼尔的治疗效果，两种批准的IPF治疗药物，与MAVS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.