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https://hdl.handle.net/20.500.14094/0100495953

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メタデータID
0100495953
アクセス権
open access
出版タイプ
Version of Record
タイトル
Revealing the UV response of melanocytes in xeroderma pigmentosum group A using patient-derived induced pluripotent stem cells
著者
Takemori, Chihiro ; Koyanagi-Aoi, Michiyo ; Fukumoto, Takeshi ; Kunisada, Makoto ; Wakamatsu, Kazumasa ; Ito, Shosuke ; Hosaka, Chieko ; Takeuchi, Seiji ; Kubo, Akiharu ; Aoi, Takashi ; Nishigori, Chikako
著者名
Takemori, Chihiro
著者ID
A0443
研究者ID
1000090432327
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail.html?systemId=5963935ba79ebd9a520e17560c007669
著者名
Koyanagi-Aoi, Michiyo
青井, 三千代
アオイ, ミチヨ
所属機関名
医学研究科
著者ID
A0865
研究者ID
1000080778770
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail.html?systemId=d16c4a96a87bb48a520e17560c007669
著者名
Fukumoto, Takeshi
福本, 毅
フクモト, タケシ
所属機関名
医学研究科
著者ID
A1477
研究者ID
1000080566969
著者名
Kunisada, Makoto
国定, 充
クニサダ, マコト
所属機関名
医学研究科
著者名
Wakamatsu, Kazumasa
著者名
Ito, Shosuke
著者名
Hosaka, Chieko
著者名
Takeuchi, Seiji
著者ID
A3081
研究者ID
1000070335256
ORCID
0000-0003-0902-3586
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail.html?systemId=6f96cd708027f025520e17560c007669
著者名
Kubo, Akiharu
久保, 亮治
クボ, アキハル
所属機関名
医学研究科
著者ID
A0444
研究者ID
1000000546997
KUID
https://kuid-rm-web.ofc.kobe-u.ac.jp/search/detail.html?systemId=fc332006706c5b9e520e17560c007669
著者名
Aoi, Takashi
青井, 貴之
アオイ, タカシ
所属機関名
医学研究科
著者ID
A0395
研究者ID
1000050198454
著者名
Nishigori, Chikako
錦織, 千佳子
ニシゴリ, チカコ
所属機関名
医学研究科
言語
English (英語)
収録物名
Journal of Dermatological Science
巻(号)
115(3)
ページ
111-120
出版者
Elsevier
刊行日
2024-09-17
公開日
2025-05-27
抄録
【Background】Xeroderma pigmentosum (XP) is characterized by photosensitivity that causes pigmentary disorder and predisposition to skin cancers on sunlight-exposed areas due to DNA repair deficiency. Patients with XP group A (XP-A) develop freckle-like pigmented maculae and depigmented maculae within a year unless strict sun-protection is enforced. Although it is crucial to study pigment cells (melanocytes: MCs) as disease target cells, establishing MCs in primary cultures is challenging. 【Objective】Elucidation of the disease pathogenesis by comparison between MCs differentiated from XP-A induced pluripotent stem cells (iPSCs) and healthy control iPSCs on the response to UV irradiation. Methods】iPSCs were established from a XP-A fibroblasts and differentiated into MCs. Differences in gene expression profiles between XP-A-iPSC-derived melanocytes (XP-A-iMCs) and Healthy control iPSC-derived MCs (HC-iMCs) were analyzed 4 and 12 h after irradiation with 30 or 150 J/m2 of UV-B using microarray analysis. 【Results】XP-A-iMCs expressed SOX10, MITF, and TYR, and showed melanin synthesis. Further, XP-A-iMCs showed reduced DNA repair ability. Gene expression profile between XP-A-iMCs and HC-iMCs revealed that, numerous gene probes that were specifically upregulated or downregulated in XP-A-iMCs after 150-J/m2 of UV-B irradiation did not return to basal levels. Of note that apoptotic pathways were highly upregulated at 150 J/m2 UV exposure in XP-A-iMCs, and cytokine-related pathways were upregulated even at 30 J/m2 UV exposure. 【Conclusion】We revealed for the first time that cytokine-related pathways were upregulated even at low-dose UV exposure in XP-A-iMCs. Disease-specific iPSCs are useful to elucidate the disease pathogenesis and develop treatment strategies of XP.
キーワード
Xeroderma pigmentosum
iPS cells
Melanocytes
Ultraviolet radiation
Microarray
DNA repair
カテゴリ
医学研究科
学術雑誌論文
権利
© 2025 The Authors.
CC license
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
関連情報
DOI
https://doi.org/10.1016/j.jdermsci.2024.06.004