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dc.contributor.author | Terada, Yukinori | en |
dc.contributor.author | Jo, Norihide | en |
dc.contributor.author | Arakawa, Yoshiki | en |
dc.contributor.author | Sakakura, Megumi | en |
dc.contributor.author | Yamada, Yosuke | en |
dc.contributor.author | Ukai, Tomoyo | en |
dc.contributor.author | Kabata, Mio | en |
dc.contributor.author | Mitsunaga, Kanae | en |
dc.contributor.author | Mineharu, Yohei | en |
dc.contributor.author | Ohta, Sho | en |
dc.contributor.author | Nakagawa, Masato | en |
dc.contributor.author | Miyamoto, Susumu | en |
dc.contributor.author | Yamamoto, Takuya | en |
dc.contributor.author | Yamada, Yasuhiro | en |
dc.contributor.alternative | 寺田, 行範 | ja |
dc.contributor.alternative | 城, 憲秀 | ja |
dc.contributor.alternative | 荒川, 芳輝 | ja |
dc.contributor.alternative | 坂倉, 恵 | ja |
dc.contributor.alternative | 山田, 洋介 | ja |
dc.contributor.alternative | 鵜飼, 智代 | ja |
dc.contributor.alternative | 蒲田, 未央 | ja |
dc.contributor.alternative | 光永, 佳奈枝 | ja |
dc.contributor.alternative | 峰晴, 陽平 | ja |
dc.contributor.alternative | 太田, 翔 | ja |
dc.contributor.alternative | 中川, 誠人 | ja |
dc.contributor.alternative | 宮本, 享 | ja |
dc.contributor.alternative | 山本, 拓也 | ja |
dc.contributor.alternative | 山田, 泰広 | ja |
dc.date.accessioned | 2019-03-06T05:43:12Z | - |
dc.date.available | 2019-03-06T05:43:12Z | - |
dc.date.issued | 2019-03-05 | - |
dc.identifier.issn | 2211-1247 | - |
dc.identifier.uri | http://hdl.handle.net/2433/236705 | - |
dc.description | ヒトiPS細胞を使った小児脳腫瘍の病態解明 --新しい治療標的を同定--. 京都大学プレスリリース. 2019-03-06. | ja |
dc.description.abstract | Atypical teratoid/rhabdoid tumor (AT/RT), which harbors SMARCB1 mutation and exhibits a characteristic histology of rhabdoid cells, has a poor prognosis because of the lack of effective treatments. Here, we establish human SMARCB1-deficient pluripotent stem cells (hPSCs). SMARCB1-deficient hPSC-derived neural progenitor-like cells (NPLCs) efficiently give rise to brain tumors when transplanted into the mouse brain. Notably, activation of an embryonic stem cell (ESC)-like signature confers a rhabdoid histology in SMARCB1-deficient NPLC-derived tumors and causes a poor prognosis. Consistently, we find the activation of the ESC-like gene expression signature and an ESC-like DNA methylation landscape in clinical specimens of AT/RT. Finally, we identify candidate genes that maintain the activation of the ESC-like signature and the growth of AT/RT cells. Collectively, SMARCB1-deficient hPSCs offer the human models for AT/RT, which uncover the role of the activated ESC-like signature in the poor prognosis and unique histology of AT/RT. | en |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Elsevier BV | en |
dc.rights | © 2019 The Author(s). This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). | en |
dc.subject | atypical teratoid/rhabdoid tumor | en |
dc.subject | pediatric tumor | en |
dc.subject | embryonic stem cell | en |
dc.subject | induced pluripotent stem cell | en |
dc.subject | ESC-like signature | en |
dc.subject | pluripotency | en |
dc.subject | dedifferentiation | en |
dc.subject | SMARCB1 | en |
dc.title | Human Pluripotent Stem Cell-Derived Tumor Model Uncovers the Embryonic Stem Cell Signature as a Key Driver in Atypical Teratoid/Rhabdoid Tumor | en |
dc.type | journal article | - |
dc.type.niitype | Journal Article | - |
dc.identifier.jtitle | Cell Reports | en |
dc.identifier.volume | 26 | - |
dc.identifier.issue | 10 | - |
dc.identifier.spage | 2608 | - |
dc.identifier.epage | 2621.e6 | - |
dc.relation.doi | 10.1016/j.celrep.2019.02.009 | - |
dc.textversion | publisher | - |
dc.identifier.pmid | 30840885 | - |
dc.relation.url | https://www.kyoto-u.ac.jp/ja/research-news/2019-03-06 | - |
dcterms.accessRights | open access | - |
datacite.awardNumber | 18H04026 | - |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName.alternative | Japan Society for the Promotion of Science (JSPS) | en |
出現コレクション: | 学術雑誌掲載論文等 |
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