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j.stemcr.2018.04.004.pdf | 4.54 MB | Adobe PDF | 見る/開く |
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dc.contributor.author | Honda-Ozaki, Fumiko | en |
dc.contributor.author | Terashima, Madoka | en |
dc.contributor.author | Niwa, Akira | en |
dc.contributor.author | Saiki, Norikazu | en |
dc.contributor.author | Kawasaki, Yuri | en |
dc.contributor.author | Ito, Haruna | en |
dc.contributor.author | Hotta, Akitsu | en |
dc.contributor.author | Nagahashi, Ayako | en |
dc.contributor.author | Igura, Koichi | en |
dc.contributor.author | Asaka, Isao | en |
dc.contributor.author | Li, Hongmei Lisa | en |
dc.contributor.author | Yanagimachi, Masakatsu | en |
dc.contributor.author | Furukawa, Fukumi | en |
dc.contributor.author | Kanazawa, Nobuo | en |
dc.contributor.author | Nakahata, Tatsutoshi | en |
dc.contributor.author | Saito, Megumu K. | en |
dc.contributor.alternative | 尾﨑(本田), 富美子 | ja |
dc.contributor.alternative | 寺嶋, 聖佳 | ja |
dc.contributor.alternative | 丹羽, 明 | ja |
dc.contributor.alternative | 佐伯, 憲和 | ja |
dc.contributor.alternative | 川崎, ゆり | ja |
dc.contributor.alternative | 堀田, 秋津 | ja |
dc.contributor.alternative | 浅香, 勲 | ja |
dc.contributor.alternative | 中畑, 龍俊 | ja |
dc.contributor.alternative | 斎藤, 潤 | ja |
dc.date.accessioned | 2018-07-31T00:54:06Z | - |
dc.date.available | 2018-07-31T00:54:06Z | - |
dc.date.issued | 2018-06-05 | - |
dc.identifier.issn | 2213-6711 | - |
dc.identifier.uri | http://hdl.handle.net/2433/233014 | - |
dc.description.abstract | Nakajo-Nishimura syndrome (NNS) is an immunoproteasome-associated autoinflammatory disorder caused by a mutation of the PSMB8 gene. Although dysfunction of the immunoproteasome causes various cellular stresses attributed to the overproduction of inflammatory cytokines and chemokines in NNS, the underlying mechanisms of the autoinflammation are still largely unknown. To investigate and understand the mechanisms and signal pathways in NNS, we established a panel of isogenic pluripotent stem cell (PSC) lines with PSMB8 mutation. Activity of the immunoproteasome in PSMB8-mutant PSC-derived myeloid cell lines (MT-MLs) was reduced even without stimulation compared with non-mutant-MLs. In addition, MT-MLs showed an overproduction of inflammatory cytokines and chemokines, with elevated reactive oxygen species (ROS) and phosphorylated p38 MAPK levels. Treatment with p38 MAPK inhibitor and antioxidants decreased the abnormal production of cytokines and chemokines. The current PSC model revealed a specific ROS-mediated inflammatory pathway, providing a platform for the discovery of alternative therapeutic options for NNS and related immunoproteasome disorders. | en |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Elsevier BV | en |
dc.rights | © 2018 The Authors. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). | en |
dc.subject | Nakajo-Nishimura syndrome (NNS) | en |
dc.subject | proteasome subunit beta type 8 (PSMB8) | en |
dc.subject | disease-specific induced pluripotent stem cells | en |
dc.subject | reactive oxygen species (ROS) | en |
dc.subject | myeloid cells | en |
dc.title | Pluripotent Stem Cell Model of Nakajo-Nishimura Syndrome Untangles Proinflammatory Pathways Mediated by Oxidative Stress | en |
dc.type | journal article | - |
dc.type.niitype | Journal Article | - |
dc.identifier.jtitle | Stem cell reports | en |
dc.identifier.volume | 10 | - |
dc.identifier.issue | 6 | - |
dc.identifier.spage | 1835 | - |
dc.identifier.epage | 1850 | - |
dc.relation.doi | 10.1016/j.stemcr.2018.04.004 | - |
dc.textversion | publisher | - |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Life Science Frontiers, Center for iPS Cell Research and Application | en |
dc.address | Department of Fundamental Cell Technology, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Fundamental Cell Technology, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Fundamental Cell Technology, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Life Science Frontiers, Center for iPS Cell Research and Application, Kyoto University・Boston Children's Hospital, Harvard Medical School | en |
dc.address | Department of Pediatrics, Tokyo Medical and Dental University | en |
dc.address | Department of Dermatology, Wakayama Medical University | en |
dc.address | Department of Dermatology, Wakayama Medical University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.address | Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University | en |
dc.identifier.pmid | 29731430 | - |
dcterms.accessRights | open access | - |
datacite.awardNumber | 13389802 | - |
datacite.awardNumber | 14431432 | - |
datacite.awardNumber | 15549896 | - |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName | 日本学術振興会 | ja |
jpcoar.funderName.alternative | Japan Society for the Promotion of Science (JSPS) | en |
jpcoar.funderName.alternative | Japan Society for the Promotion of Science (JSPS) | en |
jpcoar.funderName.alternative | Japan Society for the Promotion of Science (JSPS) | en |
出現コレクション: | 学術雑誌掲載論文等 |
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