A Mouse Model of the Most Aggressive Subgroup of Human Medulloblastoma
Ces deux études décrivent un modèle murin de glioblastome surexprimant le gène Myc
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. º Myc overexpression and Trp53 loss induce large cell anaplastic medulloblastoma º The transcriptomes of human and mouse MYC-subgroup medulloblastoma are similar º MYC-subgroup medulloblastomas are resistant to blockade of Sonic Hedgehog signaling
Cancer cell , article en libre accès, 2011