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タイトル: | Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes |
著者: | Kondo, Takayuki Funayama, Misato Miyake, Michiyo Tsukita, Kayoko Era, Takumi Osaka, Hitoshi Ayaki, Takashi Takahashi, Ryosuke https://orcid.org/0000-0002-1407-9640 (unconfirmed) Inoue, Haruhisa https://orcid.org/0000-0003-4736-9537 (unconfirmed) |
著者名の別形: | 髙橋, 良輔 井上, 治久 |
キーワード: | Alexander disease (AxD) Glial fibrillary acidic protein (GFAP) Induced pluripotent stem cells (iPSCs) Disease modeling Astrocytes Rosenthal fibers Heat-shock protein Alpha-crystallin Cytokine Inflammatory response Inherited astrocytopathy |
発行日: | 11-Jul-2016 |
出版者: | BioMed Central Ltd. |
誌名: | Acta neuropathologica communications |
巻: | 4 |
論文番号: | 69 |
抄録: | Alexander disease is a fatal neurological illness characterized by white-matter degeneration and formation of Rosenthal fibers, which contain glial fibrillary acidic protein as astrocytic inclusion. Alexander disease is mainly caused by a gene mutation encoding glial fibrillary acidic protein, although the underlying pathomechanism remains unclear. We established induced pluripotent stem cells from Alexander disease patients, and differentiated induced pluripotent stem cells into astrocytes. Alexander disease patient astrocytes exhibited Rosenthal fiber-like structures, a key Alexander disease pathology, and increased inflammatory cytokine release compared to healthy control. These results suggested that Alexander disease astrocytes contribute to leukodystrophy and a variety of symptoms as an inflammatory source in the Alexander disease patient brain. Astrocytes, differentiated from induced pluripotent stem cells of Alexander disease, could be a cellular model for future translational medicine. |
著作権等: | © 2016 The Author(s). This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided 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. |
URI: | http://hdl.handle.net/2433/216212 |
DOI(出版社版): | 10.1186/s40478-016-0337-0 |
PubMed ID: | 27402089 |
出現コレクション: | 学術雑誌掲載論文等 |
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