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.

journal_name

Cancer Cell

journal_title

Cancer cell

authors

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

doi

10.1016/j.ccr.2011.12.021

subject

Has Abstract

pub_date

2012-02-14 00:00:00

pages

155-67

issue

2

eissn

1535-6108

issn

1878-3686

pii

S1535-6108(11)00483-1

journal_volume

21

pub_type

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