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A mouse model of the most aggressive subgroup of human medulloblastoma.

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.

Pubmed ID: 22340591

Authors

  • Kawauchi D
  • Robinson G
  • Uziel T
  • Gibson P
  • Rehg J
  • Gao C
  • Finkelstein D
  • Qu C
  • Pounds S
  • Ellison DW
  • Gilbertson RJ
  • Roussel MF

Journal

Cancer cell

Publication Data

February 14, 2012

Associated Grants

  • Agency: NCI NIH HHS, Id: CA-096832
  • Agency: NCI NIH HHS, Id: CA-21765
  • Agency: NCI NIH HHS, Id: P01 CA096832
  • Agency: NCI NIH HHS, Id: P01 CA096832-08
  • Agency: NCI NIH HHS, Id: R01 CA129541

Mesh Terms

  • Animals
  • Cell Proliferation
  • Cell Transformation, Neoplastic
  • Cerebellar Neoplasms
  • Disease Models, Animal
  • Gene Expression Regulation, Neoplastic
  • Hedgehog Proteins
  • Humans
  • Medulloblastoma
  • Mice
  • Salivary alpha-Amylases
  • Transcriptome
  • Tumor Suppressor Protein p53
  • Veratrum Alkaloids