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A Mouse Model of the Most Aggressive Subgroup of Human Medulloblastoma

  作者 Kawauchi, D; Robinson, G; Uziel, T; Gibson, P; Rehg, J; Gao, CL; Finkelstein, D; Qu, CX; Pounds, S; Ellison, DW; Gilbertson, RJ; Roussel, MF  
  选自 期刊  Cancer Cell;  卷期  2012年21-2;  页码  168-180  
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[摘要]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 medulloblastonna 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.

 
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