کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
10823376 1061835 2013 7 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
ATM-deficient human neural stem cells as an in vitro model system to study neurodegeneration
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
پیش نمایش صفحه اول مقاله
ATM-deficient human neural stem cells as an in vitro model system to study neurodegeneration
چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: DNA Repair - Volume 12, Issue 8, August 2013, Pages 605-611
نویسندگان
, , , ,