Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability

Ataxia telangiectasia is a neurodegenerative inherited disease with chromosomal instability and hypersensitivity to ionizing radiation. iPS cells lacking ATM (AT-iPS cells) exhibited hypersensitivity to X-ray irradiation, one of the characteristics of the disease. While parental ataxia telangiectasi...

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Autores principales: Fukawatase, Yoshihiro, Toyoda, Masashi, Okamura, Kohji, Nakamura, Ken-ichi, Nakabayashi, Kazuhiko, Takada, Shuji, Yamazaki-Inoue, Mayu, Masuda, Akira, Nasu, Michiyo, Hata, Kenichiro, Hanaoka, Kazunori, Higuchi, Akon, Takubo, Kaiyo, Umezawa, Akihiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2014
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4073166/
https://www.ncbi.nlm.nih.gov/pubmed/24970375
http://dx.doi.org/10.1038/srep05421
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author Fukawatase, Yoshihiro
Toyoda, Masashi
Okamura, Kohji
Nakamura, Ken-ichi
Nakabayashi, Kazuhiko
Takada, Shuji
Yamazaki-Inoue, Mayu
Masuda, Akira
Nasu, Michiyo
Hata, Kenichiro
Hanaoka, Kazunori
Higuchi, Akon
Takubo, Kaiyo
Umezawa, Akihiro
author_facet Fukawatase, Yoshihiro
Toyoda, Masashi
Okamura, Kohji
Nakamura, Ken-ichi
Nakabayashi, Kazuhiko
Takada, Shuji
Yamazaki-Inoue, Mayu
Masuda, Akira
Nasu, Michiyo
Hata, Kenichiro
Hanaoka, Kazunori
Higuchi, Akon
Takubo, Kaiyo
Umezawa, Akihiro
author_sort Fukawatase, Yoshihiro
collection PubMed
description Ataxia telangiectasia is a neurodegenerative inherited disease with chromosomal instability and hypersensitivity to ionizing radiation. iPS cells lacking ATM (AT-iPS cells) exhibited hypersensitivity to X-ray irradiation, one of the characteristics of the disease. While parental ataxia telangiectasia cells exhibited significant chromosomal abnormalities, AT-iPS cells did not show any chromosomal instability in vitro for at least 80 passages (560 days). Whole exome analysis also showed a comparable nucleotide substitution rate in AT-iPS cells. Taken together, these data show that ATM is involved in protection from irradiation-induced cell death.
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spelling pubmed-40731662014-06-30 Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability Fukawatase, Yoshihiro Toyoda, Masashi Okamura, Kohji Nakamura, Ken-ichi Nakabayashi, Kazuhiko Takada, Shuji Yamazaki-Inoue, Mayu Masuda, Akira Nasu, Michiyo Hata, Kenichiro Hanaoka, Kazunori Higuchi, Akon Takubo, Kaiyo Umezawa, Akihiro Sci Rep Article Ataxia telangiectasia is a neurodegenerative inherited disease with chromosomal instability and hypersensitivity to ionizing radiation. iPS cells lacking ATM (AT-iPS cells) exhibited hypersensitivity to X-ray irradiation, one of the characteristics of the disease. While parental ataxia telangiectasia cells exhibited significant chromosomal abnormalities, AT-iPS cells did not show any chromosomal instability in vitro for at least 80 passages (560 days). Whole exome analysis also showed a comparable nucleotide substitution rate in AT-iPS cells. Taken together, these data show that ATM is involved in protection from irradiation-induced cell death. Nature Publishing Group 2014-06-27 /pmc/articles/PMC4073166/ /pubmed/24970375 http://dx.doi.org/10.1038/srep05421 Text en Copyright © 2014, Macmillan Publishers Limited. All rights reserved http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder in order to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
spellingShingle Article
Fukawatase, Yoshihiro
Toyoda, Masashi
Okamura, Kohji
Nakamura, Ken-ichi
Nakabayashi, Kazuhiko
Takada, Shuji
Yamazaki-Inoue, Mayu
Masuda, Akira
Nasu, Michiyo
Hata, Kenichiro
Hanaoka, Kazunori
Higuchi, Akon
Takubo, Kaiyo
Umezawa, Akihiro
Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title_full Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title_fullStr Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title_full_unstemmed Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title_short Ataxia telangiectasia derived iPS cells show preserved x-ray sensitivity and decreased chromosomal instability
title_sort ataxia telangiectasia derived ips cells show preserved x-ray sensitivity and decreased chromosomal instability
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4073166/
https://www.ncbi.nlm.nih.gov/pubmed/24970375
http://dx.doi.org/10.1038/srep05421
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