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Altered tau isoform ratio caused by loss of FUS and SFPQ function leads to FTLD-like phenotypes
https://repo.qst.go.jp/records/47794
https://repo.qst.go.jp/records/47794bff5ec46-26d8-4ab9-993d-ef74d9ec8523
Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2017-04-26 | |||||
タイトル | ||||||
タイトル | Altered tau isoform ratio caused by loss of FUS and SFPQ function leads to FTLD-like phenotypes | |||||
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 | |||||
アクセス権 | ||||||
アクセス権 | metadata only access | |||||
アクセス権URI | http://purl.org/coar/access_right/c_14cb | |||||
著者 |
Ishigaki, Shinsuke
× Ishigaki, Shinsuke× Fujioka, Yusuke× Okada, Yohei× Riku, Yuichi× Udagawa, Tsuyoshi× Honda, Daiyu× Yokoi, Satoshi× Endo, Kuniyuki× Ikenaka, Kensuke× Takagi, Shinnosuke× Iguchi, Yohei× Sahara, Naruhiko× Takashima, Akihiko× Okano, Hideyuki× Yoshida, Mari× Warita, Hitoshi× Aoki, Masashi× Watanabe, Hirohisa× Okado, Haruo× Katsuno, Masahisa× Sobue, Gen× 佐原 成彦 |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | Fused in sarcoma (FUS) and splicing factor, prolineand glutamine-rich (SFPQ) are RNA binding proteins that regulate RNA metabolism. We found that alternative splicing of the Mapt gene at exon 10, which generates 4-repeat tau (4R-T) and 3-repeat tau (3R-T), is regulated by interactions between FUS and SFPQ in the nuclei of neurons. Hippocampusspecific FUS- or SFPQ-knockdown mice exhibit frontotemporal lobar degeneration (FTLD)-like behaviors, reduced adult neurogenesis, accumulation of phosphorylated tau, and hippocampal atrophy with neuronal loss through an increased 4R-T/3R-T ratio. Normalization of this increased ratio by 4R-Tspecific silencing results in recovery of the normal phenotype. These findings suggest a biological link among FUS/SFPQ, tau isoform alteration, and phenotypic expression, which may function in the early pathomechanism of FTLD. |
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書誌情報 |
Cell reports 巻 18, 号 5, p. 1118-1131, 発行日 2017-01 |
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出版者 | ||||||
出版者 | Cell Press | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 2211-1247 | |||||
PubMed番号 | ||||||
識別子タイプ | PMID | |||||
関連識別子 | 28147269 | |||||
DOI | ||||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1016/j.celrep.2017.01.013 |