ATM-deficient human neural stem cells as an in vitro model system to study neurodegeneration
| Autor: | Elena Fusar Poli, Domenico Delia, Luigi Carlessi, Lidia De Filippis |
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| Rok vydání: | 2013 |
| Předmět: |
Nervous system
DNA damage Cellular differentiation Ataxia Telangiectasia Mutated Proteins Biology DNA damage response Biochemistry Nervous System Article Ataxia Telangiectasia medicine Animals Humans Neurodegeneration Induced pluripotent stem cell Hypoxia Molecular Biology Genetics Neurons Neural stem cells Cell Differentiation Neurodegenerative Diseases Cell Biology medicine.disease Neural stem cell Disease Models Animal Oxidative Stress iPS cells medicine.anatomical_structure ATM Ataxia-telangiectasia Neuroscience DNA Damage |
| Zdroj: | DNA Repair |
| ISSN: | 1568-7856 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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