ID | 117768 |
Author |
Izumikawa, Keiichi
Tokyo University of Agriculture and Technology|Japan Science and Technology Agency
Nobe, Yuko
Japan Science and Technology Agency|Tokyo Metropolitan University
Yoshikawa, Harunori
Tokyo University of Agriculture and Technology|Japan Science and Technology Agency|University of Dundee
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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
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Content Type |
Journal Article
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Description | 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.
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Journal Title |
Scientific Reports
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ISSN | 20452322
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Publisher | Springer Nature
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Volume | 7
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Start Page | 7709
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Published Date | 2017-08-09
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Rights | 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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DOI (Published Version) | |
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language |
eng
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Publisher
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departments |
Institute of Advanced Medical Sciences
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