| アイテムタイプ |
itemtype_ver1(1) |
| 公開日 |
2024-07-05 |
| タイトル |
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|
タイトル |
Simplified drug efficacy evaluation system for vasopressin neurodegenerative disease using mouse disease-specific induced pluripotent stem cells |
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言語 |
en |
| 著者 |
Miwata, Tsutomu
Suga, Hidetaka
Mitsumoto, Kazuki
Zhang, Jun
Hamada, Yoshimasa
Sakakibara, Mayu
Soen, Mika
Ozaki, Hajime
Asano, Tomoyoshi
Miyata, Takashi
Kawaguchi, Yohei
Yasuda, Yoshinori
Kobayashi, Tomoko
Sugiyama, Mariko
Onoue, Takeshi
Hagiwara, Daisuke
Iwama, Shintaro
Oyadomari, Seiichi
Arima, Hiroshi
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| アクセス権 |
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|
アクセス権 |
open access |
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アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
| 権利 |
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|
権利情報 |
© 2024. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ |
|
言語 |
en |
| 内容記述 |
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|
内容記述タイプ |
Abstract |
|
内容記述 |
Familial neurohypophyseal diabetes insipidus (FNDI) is a degenerative disorder in which vasopressin-secreting neurons degenerate over time due to the production of mutant proteins. We have demonstrated therapeutic effects of chemical chaperones in an FNDI mouse model, but the complexity and length of this evaluation were problematic. In this study, we established disease-specific mouse induced pluripotent stem cells (iPSCs) from FNDI-model mice and differentiated vasopressin neurons that produced mutant proteins. Fluorescence immunostaining showed that chemical chaperones appeared to protect vasopressin neurons generated from iPSCs derived from FNDI-model mice. Although KCL stimulation released vasopressin hormone from vasopressin neurons generated from FNDI-derived iPSCs, vasopressin hormone levels did not differ significantly between baseline and chaperone-added culture. Semi-quantification of vasopressin carrier protein and mutant protein volumes in vasopressin neurons confirmed that chaperones exerted a therapeutic effect. This research provides fundamental technology for creating in vitro disease models using human iPSCs and can be applied to therapeutic evaluation of various degenerative diseases that produce abnormal proteins. |
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言語 |
en |
| 出版者 |
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出版者 |
Elsevier |
|
言語 |
en |
| 言語 |
|
|
言語 |
eng |
| 資源タイプ |
|
|
資源タイプresource |
http://purl.org/coar/resource_type/c_6501 |
|
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 |
| 関連情報 |
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|
関連タイプ |
isVersionOf |
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|
識別子タイプ |
DOI |
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|
関連識別子 |
https://doi.org/10.1016/j.peptides.2024.171151 |
| 収録物識別子 |
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収録物識別子タイプ |
PISSN |
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収録物識別子 |
01969781 |
| 書誌情報 |
en : Peptides
巻 173,
p. 171151,
発行日 2024-03
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| ファイル公開日 |
|
|
日付 |
2025-03-01 |
|
日付タイプ |
Available |
FNDI_manuscript_20231218.pdf (1.1 MB)
