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タイトル: | TDP-43 stabilises the processing intermediates of mitochondrial transcripts |
著者: | Izumikawa, Keiichi Nobe, Yuko Yoshikawa, Harunori Ishikawa, Hideaki Miura, Yutaka Nakayama, Hiroshi Nonaka, Takashi Hasegawa, Masato Egawa, Naohiro Inoue, Haruhisa https://orcid.org/0000-0003-4736-9537 (unconfirmed) Nishikawa, Kouki Yamano, Koji Simpson, Richard J. Taoka, Masato Yamauchi, Yoshio Isobe, Toshiaki Takahashi, Nobuhiro |
著者名の別形: | 江川, 斉宏 井上, 治久 |
キーワード: | Mitochondria RNA editing |
発行日: | 9-Aug-2017 |
出版者: | Springer Nature |
誌名: | Scientific Reports |
巻: | 7 |
論文番号: | 7709 |
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. |
著作権等: | © The Author(s) 2017 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. |
URI: | http://hdl.handle.net/2433/227897 |
DOI(出版社版): | 10.1038/s41598-017-06953-y |
PubMed ID: | 28794432 |
出現コレクション: | 学術雑誌掲載論文等 |
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