人类成神经管细胞瘤侵袭性最强亚组的小鼠模型。
A mouse model of the most aggressive subgroup of human medulloblastoma.
机构信息
Department of Tumor Cell Biology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
出版信息
Cancer Cell. 2012 Feb 14;21(2):168-80. doi: 10.1016/j.ccr.2011.12.023.
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- 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.
摘要
表达大细胞/间变形态并过表达细胞 c-MYC 基因的髓母细胞瘤具有高度侵袭性，预后极差。这种所谓的 MYC 亚组在组织病理学、基因表达谱和临床行为上与其他形式的髓母细胞瘤不同。我们通过 Myc 转导 Trp53 缺失的小脑祖细胞，建立了 MYC 亚组髓母细胞瘤的小鼠模型。这些小鼠髓母细胞瘤的主要特征与人类 MYC 亚组肿瘤非常相似，与 Sonic-Hedgehog 和 WNT 疾病亚组的小鼠模型显著不同。这种小鼠模型应该会显著加速对最具侵袭性形式的髓母细胞瘤的理解和治疗，并推断出 MYC 和 MYCN 在肿瘤发生中的不同作用。
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