@article{cc06eab7313248178ea34d0a7a4d94e1,
title = "An Animal Model of MYC-Driven Medulloblastoma",
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.",
author = "Yanxin Pei and Moore, {Colin E.} and Jun Wang and Tewari, {Alok K.} and Alexey Eroshkin and Cho, {Yoon Jae} and Hendrik Witt and Andrey Korshunov and Read, {Tracy Ann} and Sun, {Julia L.} and Schmitt, {Earlene M.} and Miller, {C. Ryan} and Buckley, {Anne F.} and McLendon, {Roger E.} and Westbrook, {Thomas F.} and Northcott, {Paul A.} and Taylor, {Michael D.} and Pfister, {Stefan M.} and Febbo, {Phillip G.} and Wechsler-Reya, {Robert J.}",
note = "Funding Information: We dedicate this paper to the memory of Cameron Jackson. In addition, we would like to thank Jack Dutton and Adriana Charbono for assistance with animal colony maintenance and screening; Beth Harvat, Lynn Martinek, Mike Cook, Amy Cortez, and Yoav Altman for help with flow cytometry; Zhengzheng Wei for processing and analysis of microarrays; Irina Leiss for performing immunohistochemistry; and Daisuke Kawauchi and Martine Roussel for helpful discussions. This work was supported by funds from the Alexander and Margaret Stewart Trust and the Duke Comprehensive Cancer Center (R.W.R.), the Southeastern Brain Tumor Foundation (R.W.R.), Alex's Lemonade Stand Foundation (R.W.R.), Pediatric Brain Tumor Foundation (R.E.M. and R.W.R.), NCI grants CA122759 (R.W.R.) and CA159859 (M.D.T. and R.W.R.). C.R.M. is a Damon Runyon-Genentech Clinical Investigator supported in part by a grant (CI-45-09) from the Damon Runyon Cancer Research Foundation. R.W.R. is supported by a Leadership Award (LA1-01747) from the California Institute of Regenerative Medicine. ",
year = "2012",
month = feb,
day = "14",
doi = "10.1016/j.ccr.2011.12.021",
language = "English (US)",
volume = "21",
pages = "155--167",
journal = "Cancer Cell",
issn = "1535-6108",
publisher = "Cell Press",
number = "2",
}