ダウンロード数: 247
このアイテムのファイル:
ファイル | 記述 | サイズ | フォーマット | |
---|---|---|---|---|
sctm.2013-0172.pdf | 1.54 MB | Adobe PDF | 見る/開く |
タイトル: | Pluripotent Cell Models of Fanconi Anemia Identify the Early Pathological Defect in Human Hemoangiogenic Progenitors |
著者: | Suzuki, Naoya M. Niwa, Akira Yabe, Miharu Hira, Asuka Okada, Chihiro Amano, Naoki Watanabe, Akira https://orcid.org/0000-0002-4381-4229 (unconfirmed) Watanabe, Ken-ichiro Heike, Toshio Takata, Minoru https://orcid.org/0000-0002-4926-3675 (unconfirmed) Nakahata, Tatsutoshi Saito, Megumu K. |
著者名の別形: | 渡辺, 亮 平家, 俊夫 斎藤, 潤 |
キーワード: | Induced pluripotent stem cells Fanconi anemia Hematopoietic progenitors Differentiation Transcription factors |
発行日: | Apr-2015 |
出版者: | Wiley-Blackwell |
誌名: | Stem cells translational medicine |
巻: | 4 |
号: | 4 |
開始ページ: | 333 |
終了ページ: | 338 |
抄録: | Fanconi anemia (FA) is a disorder of genomic instability characterized by progressive bone marrow failure (BMF), developmental abnormalities, and an increased susceptibility to cancer. Although various consequences in hematopoietic stem/progenitor cells have been attributed to FA-BMF, the quest to identify the initial pathological event is still ongoing. To address this issue, we established induced pluripotent stem cells (iPSCs) from fibroblasts of six patients with FA and FANCA mutations. An improved reprogramming method yielded iPSC-like colonies from all patients, and iPSC clones were propagated from two patients. Quantitative evaluation of the differentiation ability demonstrated that the differentiation propensity toward the hematopoietic and endothelial lineages is already defective in early hemoangiogenic progenitors. The expression levels of critical transcription factors were significantly downregulated in these progenitors. These data indicate that the hematopoietic consequences in FA patients originate from the early hematopoietic stage and highlight the potential usefulness of iPSC technology for elucidating the pathogenesis of FA-BMF. |
著作権等: | © AlphaMed Press. This article is made available under the terms of the Creative Commons Attribution License. |
URI: | http://hdl.handle.net/2433/218477 |
DOI(出版社版): | 10.5966/sctm.2013-0172 |
PubMed ID: | 25762002 |
出現コレクション: | 学術雑誌掲載論文等 |
このリポジトリに保管されているアイテムはすべて著作権により保護されています。