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Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease.
http://hdl.handle.net/10422/00012602
http://hdl.handle.net/10422/00012602a28565bc-6ff1-4ca4-af93-eaec93fdbf12
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
s41467-019-13398-6 (9.2 MB)
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© The Author(s) 2019
This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/. |
| Item type | 学術雑誌論文 / Journal Article(1) | |||||
|---|---|---|---|---|---|---|
| 公開日 | 2019-12-16 | |||||
| タイトル | ||||||
| タイトル | Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease. | |||||
| 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 | |||||
| 著者 |
TSUKIYAMA, Tomoyuki
× TSUKIYAMA, Tomoyuki× KOBAYASHI, Kenichi× NAKAYA, Masataka× IWATANI, Chizuru× SEITA, Yasunari× TSUCHIYA, Hideaki× MATSUSHITA, Jun× KITAJIMA, Kahoru× KAWAMOTO, Ikuo× NAKAGAWA, Takahiro× FUKUDA, Koji× IWAKIRI, Teppei× IZUMI, Hiroyuki× ITAGAKI, Iori× KUME, Shinji× MAEGAWA, Hiroshi× NISHINAKAMURA, Ryuichi× NISHIO, Saori× NAKAMURA, Shinichiro× KAWAUCHI, Akihiro× EMA, Masatsugu |
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| 著者別名 |
築山, 智之
× 築山, 智之× 小林, 憲市× 中家, 雅隆× 岩谷, 千鶴× 清田, 弥寿成× 土屋, 英明× 松下, 淳× 北嶋, 郁× 河本, 育士× 中川, 孝博× 久米, 真司× 前川, 聡× 中村, 紳一郎× 河内, 明宏× 依馬, 正次 |
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| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Autosomal dominant polycystic kidney disease (ADPKD) caused by PKD1 mutations is one of the most common hereditary disorders. However, the key pathological processes underlying cyst development and exacerbation in pre-symptomatic stages remain unknown, because rodent models do not recapitulate critical disease phenotypes, including disease onset in heterozygotes. Here, using CRISPR/Cas9, we generate ADPKD models with PKD1 mutations in cynomolgus monkeys. As in humans and mice, near-complete PKD1 depletion induces severe cyst formation mainly in collecting ducts. Importantly, unlike in mice, PKD1 heterozygote monkeys exhibit cyst formation perinatally in distal tubules, possibly reflecting the initial pathology in humans. Many monkeys in these models survive after cyst formation, and cysts progress with age. Furthermore, we succeed in generating selective heterozygous mutations using allele-specific targeting. We propose that our models elucidate the onset and progression of ADPKD, which will serve as a critical basis for establishing new therapeutic strategies, including drug treatments. | |||||
| 書誌情報 |
en : Nature communications 巻 10, 号 1, p. 5517, 発行日 2019-12-11 |
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| 出版者 | ||||||
| 出版者 | Springer Nature | |||||
| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 2041-1723 | |||||
| PMID | ||||||
| 関連タイプ | isIdenticalTo | |||||
| 識別子タイプ | PMID | |||||
| 関連識別子 | 31822676 | |||||
| DOI | ||||||
| 関連タイプ | isIdenticalTo | |||||
| 識別子タイプ | DOI | |||||
| 関連識別子 | https://doi.org/10.1038/s41467-019-13398-6 | |||||
| 関連名称 | 10.1038/s41467-019-13398-6 | |||||
| 権利 | ||||||
| 権利情報 | © The Author(s) 2019 | |||||
| 著者版フラグ | ||||||
| 出版タイプ | VoR | |||||
| 出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
| 資源タイプ | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | Journal Article | |||||
