TY - T1的巢蛋白促进肺纤维化通过< / em > < em >促进回收TGF-β受体我摩根富林明——欧洲呼吸杂志乔——欧元和J - 10.1183/13993003.03721 -2020 SP - 2003721 AU -建成王盟Xiaofan赖盟Senyu姚明AU -海南陈盟Jianye Cai AU -玉龙罗盟易王盟-邱元盟Yinong黄盟-小越魏盟Boyan王盟-陆Qiying盟Yuanjun关非盟-王涛盟Shiyue李盟-安迪彭湘Y1 - 2021/01/01 UR - //www.qdcxjkg.com/content/early/2021/09/24/13993003.03721 - 2020. -抽象N2 -特发性肺纤维化(IPF)是一种进行性纤维化肺疾病的特点是异常激活扩散myofibroblasts和病理细胞外基质的重塑。先前的研究已经显示,中间丝蛋白巢蛋白在组织中扮演关键角色在不同的器官再生和伤口愈合。巢蛋白是否在IPF发病机制中扮演着一个关键的角色需要澄清。巢蛋白表达在bleomycin-treated老鼠和IPF患者肺组织确定。体外转染与巢蛋白成分向量规范TGF-β/ Smad信号。生物素酰化分析观察等离子体膜TβRI TβRI内吞作用和TβRI回收巢蛋白击倒后执行。腺相关病毒血清型6 (AAV6)介导的巢蛋白击倒评估体内。我们发现巢蛋白表达增加小鼠肺纤维化模型和IPF患者,调节蛋白质主要在肺α-SMA + myofibroblasts本地化。从力学上看,我们认为巢蛋白击倒抑制TGF-β信号通过抑制回收TβRI所需的细胞表面,Rab11巢蛋白促进TβRI回收的能力。体内,我们发现气管内的腺相关病毒血清型6管理局(AAV6)介导的巢蛋白可拆卸的显著减轻肺纤维化在多个实验小鼠模型。总之,我们的研究结果揭示了pro-fibrotic巢蛋白的功能部分通过促进Rab11-dependent回收TβRI和揭示肺纤维化治疗。FootnotesThis手稿最近发表在《欧洲呼吸杂志》上。 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. Wang has nothing to disclose.Conflict of interest: Dr. Lai has nothing to disclose.Conflict of interest: Dr. Yao has nothing to disclose.Conflict of interest: Dr. Chen has nothing to disclose.Conflict of interest: Dr. Cai has nothing to disclose.Conflict of interest: Dr. Luo has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Qiu has nothing to disclose.Conflict of interest: Dr. Huang has nothing to disclose.Conflict of interest: Dr. Wei has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Lu has nothing to disclose.Conflict of interest: Dr. Guan has nothing to disclose.Conflict of interest: Dr. Wang has nothing to disclose.Conflict of interest: Dr. Li has nothing to disclose.Conflict of interest: Dr. Xiang has nothing to disclose. ER -