期刊
DNA REPAIR
卷 12, 期 8, 页码 605-611出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.dnarep.2013.04.013
关键词
ATM; Neurodegeneration; Neural stem cells; iPS cells; DNA damage response; Hypoxia
资金
- Italian Telethon Foundation [GGP10066]
- Italian Association for Cancer Research (AIRC) [IG10248]
Loss of ATM kinase, a transducer of the DNA damage response and redox sensor, causes the neurodegenerative disorder ataxia-telangiectasia (A-T). While a great deal of progress has been made in elucidating the ATM-dependent DNA damage response (DDR) network, a key challenge remains in understanding the selective susceptibility of the nervous system to faulty DDR. Several factors appear implicated in the neurodegenerative phenotype in A-T, but which of them plays a crucial role remains unclear, especially since mouse models of A-T do not fully mirror the respective human syndrome. Therefore, a number of human neural stem cell (hNSC) systems have been developed to get an insight into the molecular mechanisms of neurodegeneration as consequence of ATM inactivation. Here we review the hNSC systems developed by us an others to model A-T. (C) 2013 Elsevier B.V. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据