title: A mouse model of the most aggressive subgroup of human medulloblastoma.
abstract: 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.
authors.affiliation: Department of Tumor Cell Biology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kawauchi
authors.forename: Giles
authors.initials: G
authors.lastname: Robinson
authors.forename: Tamar
authors.initials: T
authors.lastname: Uziel
authors.forename: Paul
authors.initials: P
authors.lastname: Gibson
authors.forename: Jerold
authors.initials: J
authors.lastname: Rehg
authors.forename: Cuilan
authors.initials: C
authors.lastname: Gao
authors.forename: David
authors.initials: D
authors.lastname: Finkelstein
authors.forename: Chunxu
authors.initials: C
authors.lastname: Qu
authors.forename: Stanley
authors.initials: S
authors.lastname: Pounds
authors.forename: David W
authors.initials: DW
authors.lastname: Ellison
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Gilbertson
authors.forename: Martine F
authors.initials: MF
authors.lastname: Roussel
mesh_terms: D000818:Animals; D049109:Cell Proliferation / Q000187:drug effects; D002471:Cell Transformation, Neoplastic / Q000187:drug effects; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000473:pathology*; D004195:Disease Models, Animal; D015972:Gene Expression Regulation, Neoplastic; D053823:Hedgehog Proteins / Q000037:antagonists & inhibitors; D006801:Humans; D008527:Medulloblastoma / Q000235:genetics / Q000473:pathology*; D051379:Mice; D055291:Salivary alpha-Amylases / Q000235:genetics* / Q000378:metabolism / Q000502:physiology; D059467:Transcriptome; D016159:Tumor Suppressor Protein p53 / Q000235:genetics; D014704:Veratrum Alkaloids / Q000494:pharmacology
chemical_list: D053823:Hedgehog Proteins; D016159:Tumor Suppressor Protein p53; D014704:Veratrum Alkaloids; C524799:Amy1 protein, mouse; D055291:Salivary alpha-Amylases; C000541:cyclopamine