A mouse model of the most aggressive subgroup of human medulloblastoma

Cancer Cell. 2012 Feb 14;21(2):168-80. doi: 10.1016/j.ccr.2011.12.023.


Medulloblastomas that display a large cell/anaplastic morphology and overexpress the cellular c-MYC gene are highly aggressive and carry a very poor prognosis. This so-called MYC-subgroup differs in its histopathology, gene expression profile, and clinical behavior from other forms of medulloblastoma. We generated a mouse model of MYC-subgroup medulloblastoma by transducing Trp53-null cerebellar progenitor cells with Myc. The cardinal features of these mouse medulloblastomas closely mimic those of human MYC-subgroup tumors and significantly differ from mouse models of the Sonic-Hedgehog- and WNT-disease subgroups. This mouse model should significantly accelerate understanding and treatment of the most aggressive form of medulloblastoma and infers distinct roles for MYC and MYCN in tumorigenesis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Proliferation / drug effects
  • Cell Transformation, Neoplastic / drug effects
  • Cerebellar Neoplasms / genetics
  • Cerebellar Neoplasms / pathology*
  • Disease Models, Animal
  • Gene Expression Regulation, Neoplastic
  • Hedgehog Proteins / antagonists & inhibitors
  • Humans
  • Medulloblastoma / genetics
  • Medulloblastoma / pathology*
  • Mice
  • Salivary alpha-Amylases / genetics*
  • Salivary alpha-Amylases / metabolism
  • Salivary alpha-Amylases / physiology
  • Transcriptome
  • Tumor Suppressor Protein p53 / genetics
  • Veratrum Alkaloids / pharmacology


  • Hedgehog Proteins
  • Tumor Suppressor Protein p53
  • Veratrum Alkaloids
  • Amy1 protein, mouse
  • Salivary alpha-Amylases
  • cyclopamine