Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line
Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line
复制标题
全长 TDP-43 及其 C 端片段激活 NSC34 细胞系中的线粒体自噬
DOI：
10.1016/j.neulet.2012.10.003
复制
发表时间：
2012-11-21
影响因子：
2.5
通讯作者：
Li, Chunyan
中科院分区：
文献类型：
--
作者：
Hong, Kun;Li, Yi;Li, Chunyan
TAR DNA结合蛋白43 kDa（TDP-43）与肌萎缩侧索硬化症（ALS）相关，在神经退行性疾病的发病机制中起重要作用。特别是，线粒体功能障碍参与疾病的发展。因此，我们研究了TDP-43如何与线粒体功能障碍相关。在这项研究中，我们发现TDP-43及其C-末端片段的过表达导致线粒体损伤。此外，全长TDP-43和截短的TDP-43位于线粒体中，其中自噬被激活，由LC 3-II和p62的变化指示。这些研究表明，人TDP-43及其C-末端片段可能导致线粒体功能障碍并增强线粒体自噬。(C)2012爱思唯尔爱尔兰有限公司保留所有权利。
TAR DNA binding protein of 43 kDa (TDP-43), which has been associated with amyotrophic lateral sclerosis (ALS), plays an essential role in neurodegenerative disease pathogenesis. In particular, mitochondrial dysfunction is involved in the disease development. Thus, we investigated how TDP-43 is related to mitochondrial dysfunction. In this study, we found that overexpression of TDP-43 and its C-terminal fragments resulted in mitochondrial damage. In addition, full-length TDP-43 and truncated TDP-43 were localized in the mitochondria, where autophagy was activated, indicated by changes of LC3-II and p62. These studies suggest that human TDP-43 and its C-terminal fragments may cause mitochondrial dysfunction and enhance mitophagy. (C) 2012 Elsevier Ireland Ltd. All rights reserved.