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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Detalles Bibliográficos
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
Descripción
Sumario: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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