An animal model of MYC-driven medulloblastoma.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Pei, Y; Moore, CE; Wang, J; Tewari, AK; Eroshkin, A; Cho, Y-J; Witt, H; Korshunov, A; Read, T-A; Sun, JL; Schmitt, EM; Miller, CR; Buckley, AF; McLendon, RE; Westbrook, TF; Northcott, PA; Taylor, MD; Pfister, SM; Febbo, PG; Wechsler-Reya, RJ

Published Date

  • February 14, 2012

Published In

Volume / Issue

  • 21 / 2

Start / End Page

  • 155 - 167

PubMed ID

  • 22340590

Pubmed Central ID

  • PMC3285431

Electronic International Standard Serial Number (EISSN)

  • 1878-3686

Digital Object Identifier (DOI)

  • 10.1016/j.ccr.2011.12.021


  • eng

Conference Location

  • United States