A Mouse Model of the Most Aggressive Subgroup of Human Medulloblastoma

作者：Kawauchi Daisuke; Robinson Giles; Uziel Tamar; Gibson Paul; Rehg Jerold; Gao Cuilan; Finkelstein David; Qu Chunxu; Pounds Stanley; Ellison David W; Gilbertson Richard J; Roussel Martine F^*

来源：Cancer Cell, 2012, 21(2): 168-180.

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 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.

• 出版日期2012-2-14