| Item type |
文献 / Documents(1) |
| 公開日 |
2023-09-06 |
| アクセス権 |
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アクセス権 |
open access |
| 資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
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資源タイプ |
journal article |
| 出版社版DOI |
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関連識別子 |
https://doi.org/10.1038/s41598-017-06953-y |
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関連名称 |
10.1038/s41598-017-06953-y |
| 出版タイプ |
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|
出版タイプ |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| タイトル |
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タイトル |
TDP-43 stabilises the processing intermediates of mitochondrial transcripts |
| 著者 |
イズミカワ, ケイイチ
ノベ, ユウコ
𠮷川, 治孝
イシカワ, ヒデアキ
ミウラ, ユタカ
ナカヤマ, ヒロシ
ノナカ, タカシ
ハセガワ, マサト
エガワ, ナオヒロ
イノウエ, ハルヒサ
ニシカワ, コウキ
ヤマノ, コウジ
Simpson, Richard J.
タオカ, マサト
ヤマウチ, ヨシオ
イソベ, トシアキ
タカハシ, ノブヒロ
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| 抄録 |
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内容記述 |
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. |
| 書誌情報 |
en : Scientific Reports
巻 7,
p. 7709,
発行日 2017-08-09
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| 収録物ID |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
20452322 |
| 出版者 |
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出版者 |
Springer Nature |
| 権利情報 |
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|
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/. |
| EID |
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識別子 |
372793 |
| 言語 |
|
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言語 |
eng |
srep_7_7709.pdf (4.02 MB)
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