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Title: Human Pluripotent Stem Cell-Derived Tumor Model Uncovers the Embryonic Stem Cell Signature as a Key Driver in Atypical Teratoid/Rhabdoid Tumor
Authors: Terada, Yukinori  kyouindb  KAKEN_id
Jo, Norihide  kyouindb  KAKEN_id
Arakawa, Yoshiki  kyouindb  KAKEN_id  orcid (unconfirmed)
Sakakura, Megumi
Yamada, Yosuke  kyouindb  KAKEN_id
Ukai, Tomoyo
Kabata, Mio
Mitsunaga, Kanae
Mineharu, Yohei  kyouindb  KAKEN_id
Ohta, Sho
Nakagawa, Masato
Miyamoto, Susumu
Yamamoto, Takuya  kyouindb  KAKEN_id
Yamada, Yasuhiro
Author's alias: 寺田, 行範
城, 憲秀
荒川, 芳輝
坂倉, 恵
山田, 洋介
鵜飼, 智代
蒲田, 未央
光永, 佳奈枝
峰晴, 陽平
太田, 翔
中川, 誠人
宮本, 享
山本, 拓也
山田, 泰広
Keywords: atypical teratoid/rhabdoid tumor
pediatric tumor
embryonic stem cell
induced pluripotent stem cell
ESC-like signature
Issue Date: 5-Mar-2019
Publisher: Elsevier BV
Journal title: Cell Reports
Volume: 26
Issue: 10
Start page: 2608
End page: 2621.e6
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.
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.
Rights: © 2019 The Author(s). This is an open access article under the CC BY license (
DOI(Published Version): 10.1016/j.celrep.2019.02.009
PubMed ID: 30840885
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