共济失调毛细血管扩张症衍生的诱导多能干细胞显示出保留的X射线敏感性和降低的染色体不稳定性。

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

作者信息

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

机构信息

1] Department of Reproductive Biology, National Research Institute for Child Health and Development, Tokyo, 157-8535, Japan [2] School of BioMedical Science, Tokyo Medical and Dental University, Tokyo, 113-0034, Japan.

1] Department of Reproductive Biology, National Research Institute for Child Health and Development, Tokyo, 157-8535, Japan [2] Department of Research Team for Geriatric Medicine, Tokyo Metropolitan Institute of Gerontology, Tokyo, 173-0015, Japan.

出版信息

Sci Rep. 2014 Jun 27;4:5421. doi: 10.1038/srep05421.

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.