| Item type |
デフォルトアイテムタイプ（フル）(1) |
| 公開日 |
2020-06-04 |
| タイトル |
|
|
タイトル |
Organoids from Nephrotic Disease-Derived iPSCs Identify Impaired NEPHRIN Localization and Slit Diaphragm Formation in Kidney Podocytes |
|
言語 |
en |
| 作成者 |
Tanigawa, Shunsuke
Islam, Mazharul
Sharmin, Sazia
Naganuma, Hidekazu
Yoshimura, Yasuhiro
Haque, Fahim
Era, Takumi
Nakazato, Hitoshi
Nakanishi, Koichi
Sakuma, Tetsushi
Yamamoto, Takashi
Kurihara, Hidetake
Taguchi, Atsuhiro
Nishinakamura, Ryuichi
|
| 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 |
| 権利情報 |
|
|
言語 |
en |
|
権利情報 |
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 |
| 権利情報 |
|
|
言語 |
en |
|
権利情報Resource |
https://creativecommons.org/licenses/by-nc-nd/4.0/ |
|
権利情報 |
https://creativecommons.org/licenses/by-nc-nd/4.0/ |
| 内容記述 |
|
|
内容記述タイプ |
Other |
|
内容記述 |
Mutations in the NPHS1 gene, which encodes NEPHRIN, cause congenital nephrotic syndrome, resulting from impaired slit diaphragm (SD) formation in glomerular podocytes. However, methods for SD reconstitution have been unavailable, thereby limiting studies in the field. In the present study, we established human induced pluripotent stem cells (iPSCs) from a patient with an NPHS1 missense mutation, and reproduced the SD formation process using iPSC-derived kidney organoids. The mutant NEPHRIN failed to become localized on the cell surface for pre-SD domain formation in the induced podocytes. Upon transplantation, the mutant podocytes developed foot processes, but exhibited impaired SD formation. Genetic correction of the single amino acid mutation restored NEPHRIN localization and phosphorylation, colocalization of other SD-associated proteins, and SD formation. Thus, these kidney organoids from patient-derived iPSCs identified SD abnormalities in the podocytes at the initial phase of congenital nephrotic disease. |
| 内容記述 |
|
|
内容記述タイプ |
Other |
|
内容記述 |
論文 |
| 出版者 |
|
|
出版者 |
Cell 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 |
VoR |
|
出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| 識別子 |
|
|
識別子 |
http://hdl.handle.net/20.500.12000/46025 |
|
識別子タイプ |
HDL |
| 関連情報 |
|
|
|
識別子タイプ |
DOI |
|
|
関連識別子 |
https://doi.org/10.1016/j.stemcr.2018.08.003 |
| 関連情報 |
|
|
|
識別子タイプ |
DOI |
|
|
関連識別子 |
https://doi.org/10.1016/j.stemcr.2018.08.003 |
| 収録物識別子 |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
2213-6711 |
| 収録物名 |
|
|
収録物名 |
Stem Cell Reports |
|
言語 |
en |
| 書誌情報 |
巻 11,
号 3,
p. 727-740,
発行日 2018-09-11
|
1-s2.0-S2213671118303242-main.pdf
