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Title: In Vitro Disease Modeling of Hermansky-Pudlak Syndrome Type 2 Using Human Induced Pluripotent Stem Cell-Derived Alveolar Organoids
Authors: Korogi, Yohei
Gotoh, Shimpei
Ikeo, Satoshi
Yamamoto, Yuki
Sone, Naoyuki
Tamai, Koji
Konishi, Satoshi
Nagasaki, Tadao
Matsumoto, Hisako  kyouindb  KAKEN_id
Ito, Isao
Chen-Yoshikawa, Toyofumi F.
Date, Hiroshi
Hagiwara, Masatoshi  kyouindb  KAKEN_id
Asaka, Isao  kyouindb  KAKEN_id
Hotta, Akitsu
Mishima, Michiaki
Hirai, Toyohiro
Author's alias: 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
Keywords: iPSC, Hermansky-Pudlak syndrome, HPS, lamellar body, alveolar type 2 cell, pulmonary surfactant, pluripotent stem cell, pulmonary fibrosis, alveolar organoid, MX35
Issue Date: 5-Mar-2019
Publisher: Elsevier BV
Journal title: Stem Cell Reports
Volume: 12
Issue: 3
Start page: 431
End page: 440
Abstract: 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.
Description: 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.
Rights: © 2019 The Author(s). This is an open access article under the CC BY license (
DOI(Published Version): 10.1016/j.stemcr.2019.01.014
PubMed ID: 30773483
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