AN ANIMAL MODEL OF MYC-DRIVEN MEDULLOBLASTOMA
| Autor: | C. Ryan Miller, Thomas F. Westbrook, Julia L. Sun, Alexey Eroshkin, Anne F. Buckley, Hendrik Witt, Tracy Ann Read, Andrey Korshunov, Roger E. McLendon, Yanxin Pei, Michael D. Taylor, Phillip G. Febbo, Alok K. Tewari, Robert J. Wechsler-Reya, Colin E. Moore, Paul A. Northcott, Earlene M. Schmitt, Jun Wang, Stefan M. Pfister, Yoon Jae Cho |
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| Jazyk: | angličtina |
| Rok vydání: | 2012 |
| Předmět: |
Medulloblastoma
0303 health sciences Cerebellum Cancer Research Oncogene Cerebellar Neoplasm Cell Biology Biology medicine.disease Hedgehog signaling pathway Neural stem cell Article 3. Good health 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure Oncology 030220 oncology & carcinogenesis medicine Cancer research Stem cell PI3K/AKT/mTOR pathway 030304 developmental biology |
| Popis: | SummaryMedulloblastoma (MB) is the most common malignant brain tumor in children. Patients whose tumors exhibit overexpression or amplification of the MYC oncogene (c-MYC) usually have an extremely poor prognosis, but there are no animal models of this subtype of the disease. Here, we show that cerebellar stem cells expressing Myc and mutant Trp53 (p53) generate aggressive tumors following orthotopic transplantation. These tumors consist of large, pleiomorphic cells and resemble human MYC-driven MB at a molecular level. Notably, antagonists of PI3K/mTOR signaling, but not Hedgehog signaling, inhibit growth of tumor cells. These findings suggest that cerebellar stem cells can give rise to MYC-driven MB and identify a novel model that can be used to test therapies for this devastating disease. |
| Databáze: | OpenAIRE |
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