ATM-deficient human neural stem cells as an in vitro model system to study neurodegeneration

Luigi Carlessi, Elena Fusar Poli, Lidia De Filippis, Domenico Delia

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)605-611
Number of pages7
JournalDNA Repair
Volume12
Issue number8
DOIs
Publication statusPublished - Aug 2013

Keywords

  • ATM
  • DNA damage response
  • Hypoxia
  • IPS cells
  • Neural stem cells
  • Neurodegeneration

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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