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ID 117768
著者
Izumikawa, Keiichi Tokyo University of Agriculture and Technology|Japan Science and Technology Agency
Nobe, Yuko Japan Science and Technology Agency|Tokyo Metropolitan University
吉川, 治孝 Tokyo University of Agriculture and Technology|Japan Science and Technology Agency|University of Dundee 徳島大学 教育研究者総覧 KAKEN研究者をさがす
Ishikawa, Hideaki Tokyo University of Agriculture and Technology|Japan Science and Technology Agency
Miura, Yutaka Tokyo University of Agriculture and Technology
Nakayama, Hiroshi RIKEN
Nonaka, Takashi Tokyo Metropolitan Institute of Medical Science
Hasegawa, Masato Tokyo Metropolitan Institute of Medical Science
Egawa, Naohiro Kyoto University
Inoue, Haruhisa Kyoto University
Nishikawa, Kouki Nagoya University
Yamano, Koji Tokyo Metropolitan Institute of Medical Sciences
Simpson, Richard J. Tokyo University of Agriculture and Technology|La Trobe University
Taoka, Masato Japan Science and Technology Agency|Tokyo Metropolitan University
Yamauchi, Yoshio Japan Science and Technology Agency|Tokyo Metropolitan University
Isobe, Toshiaki Japan Science and Technology Agency|Tokyo Metropolitan University
Takahashi, Nobuhiro Tokyo University of Agriculture and Technology|Japan Science and Technology Agency
資料タイプ
学術雑誌論文
抄録
The 43-kDa trans-activating response region DNA-binding protein 43 (TDP-43) is a product of a causative gene for amyotrophic lateral sclerosis (ALS). Despite of accumulating evidence that mitochondrial dysfunction underlies the pathogenesis of TDP-43–related ALS, the roles of wild-type TDP-43 in mitochondria are unknown. Here, we show that the small TDP-43 population present in mitochondria binds directly to a subset of mitochondrial tRNAs and precursor RNA encoded in L-strand mtDNA. Upregulated expression of TDP-43 stabilised the processing intermediates of mitochondrial polycistronic transcripts and their products including the components of electron transport and 16S mt-rRNA, similar to the phenotype observed in cells deficient for mitochondrial RNase P. Conversely, TDP-43 deficiency reduced the population of processing intermediates and impaired mitochondrial function. We propose that TDP-43 has a novel role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts.
掲載誌名
Scientific Reports
ISSN
20452322
出版者
Springer Nature
7
開始ページ
7709
発行日
2017-08-09
権利情報
This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
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言語
eng
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出版社版
部局
先端酵素学研究所