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Title: Pluripotent Cell Models of Fanconi Anemia Identify the Early Pathological Defect in Human Hemoangiogenic Progenitors
Authors: Suzuki, Naoya M.
Niwa, Akira  kyouindb  KAKEN_id
Yabe, Miharu
Hira, Asuka
Okada, Chihiro
Amano, Naoki
Watanabe, Akira  kyouindb  KAKEN_id  orcid https://orcid.org/0000-0002-4381-4229 (unconfirmed)
Watanabe, Ken-ichiro
Heike, Toshio
Takata, Minoru  kyouindb  KAKEN_id
Nakahata, Tatsutoshi
Saito, Megumu K.
Author's alias: 渡辺, 亮
平家, 俊夫
斎藤, 潤
Keywords: Induced pluripotent stem cells
Fanconi anemia
Hematopoietic progenitors
Differentiation
Transcription factors
Issue Date: Apr-2015
Publisher: Wiley-Blackwell
Journal title: Stem cells translational medicine
Volume: 4
Issue: 4
Start page: 333
End page: 338
Abstract: 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.
Rights: © AlphaMed Press. This article is made available under the terms of the Creative Commons Attribution License.
URI: http://hdl.handle.net/2433/218477
DOI(Published Version): 10.5966/sctm.2013-0172
Appears in Collections:Journal Articles

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