| Literature DB >> 22340590 |
Yanxin Pei1, Colin E Moore, Jun Wang, Alok K Tewari, Alexey Eroshkin, Yoon-Jae Cho, Hendrik Witt, Andrey Korshunov, Tracy-Ann Read, Julia L Sun, Earlene M Schmitt, C Ryan Miller, Anne F Buckley, Roger E McLendon, Thomas F Westbrook, Paul A Northcott, Michael D Taylor, Stefan M Pfister, Phillip G Febbo, Robert J Wechsler-Reya.
Abstract
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. Copyright ÂEntities:
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Year: 2012 PMID： 22340590 PMCID： PMC3285431 DOI： 10.1016/j.ccr.2011.12.021
Source DB: PubMed Journal: Cancer Cell ISSN： 1535-6108 Impact factor: 31.743