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

Yoshihiro Fukawatase, Masashi Toyoda, Kohji Okamura, Ken Ichi Nakamura, Kazuhiko Nakabayashi, Shuji Takada, Mayu Yamazaki-Inoue, Akira Masuda, Michiyo Nasu, Kenichiro Hata, Kazunori Hanaoka, Akon Higuchi, Kaiyo Takubo, Akihiro Umezawa

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

Abstract

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.

Original languageEnglish
Article number5421
JournalScientific Reports
Volume4
DOIs
StatePublished - 27 Jun 2014

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