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j.stemcr.2019.01.014.pdf | 3.32 MB | Adobe PDF | 見る/開く |
タイトル: | In Vitro Disease Modeling of Hermansky-Pudlak Syndrome Type 2 Using Human Induced Pluripotent Stem Cell-Derived Alveolar Organoids |
著者: | Korogi, Yohei Gotoh, Shimpei Ikeo, Satoshi Yamamoto, Yuki Sone, Naoyuki https://orcid.org/0000-0002-3378-728X (unconfirmed) Tamai, Koji Konishi, Satoshi Nagasaki, Tadao Matsumoto, Hisako Ito, Isao https://orcid.org/0000-0003-3109-898X (unconfirmed) Chen-Yoshikawa, Toyofumi F. Date, Hiroshi Hagiwara, Masatoshi Asaka, Isao Hotta, Akitsu https://orcid.org/0000-0002-2619-7441 (unconfirmed) Mishima, Michiaki Hirai, Toyohiro |
著者名の別形: | 興梠, 陽平 後藤, 慎平 池尾, 聡 山本, 佑樹 曽根, 尚之 玉井, 浩二 小西, 聡史 長崎, 忠雄 松本, 久子 伊藤, 功朗 陳, 豊史 伊達, 洋至 萩原, 正敏 浅香, 勲 堀田, 秋津 三嶋, 理晃 平井, 豊博 |
キーワード: | iPSC, Hermansky-Pudlak syndrome, HPS, lamellar body, alveolar type 2 cell, pulmonary surfactant, pluripotent stem cell, pulmonary fibrosis, alveolar organoid, MX35 |
発行日: | 5-Mar-2019 |
出版者: | Elsevier BV |
誌名: | Stem Cell Reports |
巻: | 12 |
号: | 3 |
開始ページ: | 431 |
終了ページ: | 440 |
抄録: | It has been challenging to generate in vitro models of alveolar lung diseases, as the stable culture of alveolar type 2 (AT2) cells has been difficult. Methods of generating and expanding AT2 cells derived from induced pluripotent stem cells (iPSCs) have been established and are expected to be applicable to disease modeling. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by dysfunction of lysosome-related organelles, such as lamellar bodies (LBs), in AT2 cells. From an HPS type 2 (HPS2) patient, we established disease-specific iPSCs (HPS2-iPSCs) and their gene-corrected counterparts. By live cell imaging, the LB dynamics were visualized and altered distribution, enlargement, and impaired secretion of LBs were demonstrated in HPS2-iPSC-derived AT2 cells. These findings provide insight into the AT2 dysfunction in HPS patients and support the potential use of human iPSC-derived AT2 cells for future research on alveolar lung diseases. |
記述: | iPS細胞を用いて遺伝性間質性肺炎の病態解析に成功 --間質性肺炎の原因究明の足がかりに--. 京都大学プレスリリース. 2019-02-19. |
著作権等: | © 2019 The Author(s). This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
URI: | http://hdl.handle.net/2433/236487 |
DOI(出版社版): | 10.1016/j.stemcr.2019.01.014 |
PubMed ID: | 30773483 |
関連リンク: | https://www.kyoto-u.ac.jp/ja/research-news/2019-02-19 |
出現コレクション: | 学術雑誌掲載論文等 |
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