A mouse model of the most aggressive subgroup of human medulloblastoma

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 medulloblastom...

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Vydáno v:Cancer cell Ročník 21; číslo 2; s. 168
Hlavní autoři: 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
Médium: Journal Article
Jazyk:angličtina
Vydáno: United States 14.02.2012
Témata:
Animals
Cell Proliferation - drug effects
Cell Transformation, Neoplastic - drug effects
Cerebellar Neoplasms - genetics
Cerebellar Neoplasms - pathology
Disease Models, Animal
Gene Expression Regulation, Neoplastic
Hedgehog Proteins - antagonists & inhibitors
Humans
Medulloblastoma - genetics
Medulloblastoma - pathology
Mice
Salivary alpha-Amylases - genetics
Salivary alpha-Amylases - metabolism
Salivary alpha-Amylases - physiology
Transcriptome
Tumor Suppressor Protein p53 - genetics
Veratrum Alkaloids - pharmacology
ISSN:1878-3686, 1878-3686
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Popis
Shrnutí: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.
Bibliografie:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:1878-3686
1878-3686
DOI:10.1016/j.ccr.2011.12.023