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Publication : An animal model of MYC-driven medulloblastoma.

First Author  Pei Y Year  2012
Journal  Cancer Cell Volume  21
Issue  2 Pages  155-67
PubMed ID  22340590 Mgi Jnum  J:181463
Mgi Id  MGI:5311487 Doi  10.1016/j.ccr.2011.12.021
Citation  Pei Y, et al. (2012) An Animal Model of MYC-Driven Medulloblastoma. Cancer Cell 21(2):155-67
abstractText  Medulloblastoma (MB) is the most common malignant brain tumor in children. Patients whose tumors exhibit overexpression or amplification of the MYC oncogene (c-MYC) usually have an extremely poor prognosis, but there are no animal models of this subtype of the disease. Here, we show that cerebellar stem cells expressing Myc and mutant Trp53 (p53) generate aggressive tumors following orthotopic transplantation. These tumors consist of large, pleiomorphic cells and resemble human MYC-driven MB at a molecular level. Notably, antagonists of PI3K/mTOR signaling, but not Hedgehog signaling, inhibit growth of tumor cells. These findings suggest that cerebellar stem cells can give rise to MYC-driven MB and identify a novel model that can be used to test therapies for this devastating disease.
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