ID | 115015 |
Author |
Oshima, Koichi
Kyoto University|Columbia University
Saiki, Norikazu
Kyoto University
Tanaka, Michihiro
Kyoto University
Imamura, Hiromi
Kyoto University
Niwa, Akira
Kyoto University
Nagahashi, Ayako
Kyoto University
Hirayama, Akiyoshi
Keio University
Okita, Keisuke
Kyoto University
Hotta, Akitsu
Kyoto University
Kitayama, Shuichi
Kyoto University
Osawa, Mitsujiro
Kyoto University
Kaneko, Shin
Kyoto University
Watanabe, Akira
Kyoto University
Asaka, Isao
Kyoto University
Fujibuchi, Wataru
Kyoto University
Imai, Kohsuke
Tokyo Medical and Dental University
Yabe, Hiromasa
Tokai University
Kamachi, Yoshiro
Nagoya University
Hara, Junichi
Osaka City General Hospital
Kojima, Seiji
Nagoya University
Tomita, Masaru
Keio University
Soga, Tomoyoshi
Keio University
Noma, Takafumi
The University of Tokushima
Tokushima University Educator and Researcher Directory
KAKEN Search Researchers
Nonoyama, Shigeaki
National Defense Medical College
Nakahata, Tatsutoshi
Kyoto University
Saito, Megumu K.
Kyoto University
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Keywords | Induced pluripotent stem cells
Adenylate kinase 2
Hemoangiogenic progenitor cells
Phosphotransfer
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Content Type |
Journal Article
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Description | AK2 is an adenylate phosphotransferase that localizes at the intermembrane spaces of the mitochondria, and its mutations cause a severe combined immunodeficiency with neutrophil maturation arrest named reticular dysgenesis (RD). Although the dysfunction of hematopoietic stem cells (HSCs) has been implicated, earlier developmental events that affect the fate of HSCs and/or hematopoietic progenitors have not been reported. Here, we used RD-patient-derived induced pluripotent stem cells (iPSCs) as a model of AK2-deficient human cells. Hematopoietic differentiation from RD-iPSCs was profoundly impaired. RD-iPSC-derived hemoangiogenic progenitor cells (HAPCs) showed decreased ATP distribution in the nucleus and altered global transcriptional profiles. Thus, AK2 has a stage-specific role in maintaining the ATP supply to the nucleus during hematopoietic differentiation, which affects the transcriptional profiles necessary for controlling the fate of multipotential HAPCs. Our data suggest that maintaining the appropriate energy level of each organelle by the intracellular redistribution of ATP is important for controlling the fate of progenitor cells.
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Journal Title |
Biochemical and Biophysical Research Communications
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ISSN | 0006291X
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NCID | AA00564395
AA11542044
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Publisher | Elsevier
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Volume | 497
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Issue | 2
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Start Page | 719
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End Page | 725
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Published Date | 2018-02-17
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Rights | © 2018 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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EDB ID | |
DOI (Published Version) | |
URL ( Publisher's Version ) | |
FullText File | |
language |
eng
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TextVersion |
Publisher
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departments |
Oral Sciences
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