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Publication : DNA ligase IV suppresses medulloblastoma formation.

First Author  Lee Y Year  2002
Journal  Cancer Res Volume  62
Issue  22 Pages  6395-9
PubMed ID  12438222 Mgi Jnum  J:80298
Mgi Id  MGI:2445637 Citation  Lee Y, et al. (2002) DNA Ligase IV Suppresses Medulloblastoma Formation. Cancer Res 62(22):6395-9
abstractText  Substantial neural defects are often present in mice with targeted inactivation of DNA repair factors such as DNA ligase IV (Lig4). Whereas Lig4(-/-) mice undergo widespread neural apoptosis and die during development, p53 deficiency rescues this death. We found that all Lig4(-/-)p53(-/-) mice developed medulloblastoma, but did not develop other tumors of the nervous system. Lig4(-/-)p53(-/-) medulloblastoma occurred as early as 21 days of age, originated in the external granule layer of the developing cerebellum, and was synaptophysin immunoreactive. These data reveal a pronounced susceptibility of the cerebellum to the effects of chronic DNA damage and provide a direct link between genotoxic stress and medulloblastoma formation.
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