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Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases
http://hdl.handle.net/2237/00033493
http://hdl.handle.net/2237/00033493060ca476-1c84-41aa-83b2-d581acdbd1fc
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
FUS_SFPQ_pathologyR1 (6.1 MB)
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| Item type | 学術雑誌論文 / Journal Article(1) | |||||
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| 公開日 | 2021-02-08 | |||||
| タイトル | ||||||
| タイトル | Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases | |||||
| 言語 | en | |||||
| 著者 |
Ishigaki, Shinsuke
× Ishigaki, Shinsuke× Riku, Yuichi× Fujioka, Yusuke× Endo, Kuniyuki× Iwade, Nobuyuki× Kawai, Kaori× Ishibashi, Minaka× Yokoi, Satoshi× Katsuno, Masahisa× Watanabe, Hirohisa× Mori, Keiko× Akagi, Akio× Yokota, Osamu× Terada, Seishi× Kawakami, Ito× Suzuki, Naoki× Warita, Hitoshi× Aoki, Masashi× Yoshida, Mari× Sobue, Gen |
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| アクセス権 | ||||||
| アクセス権 | open access | |||||
| アクセス権URI | http://purl.org/coar/access_right/c_abf2 | |||||
| 権利 | ||||||
| 言語 | en | |||||
| 権利情報 | This is a pre-copyedited, author-produced version of an article accepted for publication in [Brain] following peer review. The version of record [Brain. v.143, n.8, 2020, p.2398-2405] is available online at: http://doi.org/10.1093/brain/awaa196. | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | FTLD | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | FUS | |||||
| キーワード | ||||||
| 主題Scheme | Other | |||||
| 主題 | SFPQ | |||||
| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Fused in sarcoma (FUS) is genetically and clinicopathologically linked to frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). We have previously reported that intranuclear interactions of FUS and splicing factor, proline- and glutamine-rich (SFPQ) contribute to neuronal homeostasis. Disruption of the FUS-SFPQ interaction leads to an increase in the ratio of 4-repeat tau (4R-tau)/3-repeat tau (3R-tau), which manifests in FTLD-like phenotypes in mice. Here, we examined FUS-SFPQ interactions in 142 autopsied individuals with FUS-related ALS/FTLD (ALS/FTLD-FUS), TDP-43-related ALS/FTLD (ALS/FTLD-TDP), progressive supranuclear palsy, corticobasal degeneration, Alzheimer’s disease, or Pick’s disease as well as controls. Immunofluorescent imaging showed impaired intranuclear co-localization of FUS and SFPQ in neurons of ALS/FTLD-FUS, ALS/FTLD-TDP, progressive supranuclear palsy and corticobasal degeneration cases, but not in Alzheimer’s disease or Pick’s disease cases. Immunoprecipitation analyses of FUS and SFPQ revealed reduced interactions between the two proteins in ALS/FTLD-TDP and progressive supranuclear palsy cases, but not in those with Alzheimer disease. Furthermore, the ratio of 4R/3R-tau was elevated in cases with ALS/FTLD-TDP and progressive supranuclear palsy, but was largely unaffected in cases with Alzheimer disease. We concluded that impaired interactions between intranuclear FUS and SFPQ and the subsequent increase in the ratio of 4R/3R-tau constitute a common pathogenesis pathway in FTLD spectrum diseases. | |||||
| 言語 | en | |||||
| 内容記述 | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | ファイル公開日: 2021/08/08 | |||||
| 言語 | ja | |||||
| 出版者 | ||||||
| 出版者 | Oxford University Press | |||||
| 言語 | en | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | journal article | |||||
| 出版タイプ | ||||||
| 出版タイプ | AM | |||||
| 出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||
| DOI | ||||||
| 関連タイプ | isVersionOf | |||||
| 識別子タイプ | DOI | |||||
| 関連識別子 | https://doi.org/10.1093/brain/awaa196 | |||||
| ISSN（print） | ||||||
| 収録物識別子タイプ | PISSN | |||||
| 収録物識別子 | 0006-8950 | |||||
| 書誌情報 |
en : Brain 巻 143, 号 8, p. 2398-2405, 発行日 2020-08-08 |
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| 著者版フラグ | ||||||
| 値 | author | |||||
