1. A Mouse Model of the Most Aggressive Subgroup of Human Medulloblastoma
- Author
-
David W. Ellison, David Finkelstein, Cuilan Gao, Stanley Pounds, Giles W. Robinson, Chunxu Qu, Paul Gibson, Richard J. Gilbertson, Martine F. Roussel, Tamar Uziel, Jerold E. Rehg, and Daisuke Kawauchi
- Subjects
Cancer Research ,Biology ,medicine.disease_cause ,Article ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Progenitor cell ,neoplasms ,030304 developmental biology ,Regulation of gene expression ,Medulloblastoma ,0303 health sciences ,Large cell ,Cerebellar Neoplasm ,Cell Biology ,medicine.disease ,nervous system diseases ,Veratrum alkaloid ,stomatognathic diseases ,CXCL3 ,Oncology ,030220 oncology & carcinogenesis ,Immunology ,Cancer research ,Carcinogenesis - 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 (SHH)- 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.
- Published
- 2012
- Full Text
- View/download PDF