Article
Differential DNA damage signaling accounts for distinct neural apoptotic responses in ATLD and NBS.
Genes & development - 15 Jan 2009
Shull Erin R P, Lee Youngsoo, Nakane Hironobu, Stracker Travis H, Zhao Jingfeng, Russell Helen R, Petrini John H J, McKinnon Peter J
Abstract excerpt
The MRN complex (Mre11/RAD50/NBS1) and ATM (ataxia telangiectasia, mutated) are critical for the cellular response to DNA damage. ATM disruption causes ataxia telangiectasia (A-T), while MRN dysfunction can lead to A-T-like disease (ATLD) or Nijmegen breakage syndrome (NBS). Neuropathology is a hallmark of these diseases, whereby neurodegeneration occurs in A-T and ATLD while microcephaly characterizes NBS. To...
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