Characterization of the Ews/Fli-1 Chimeric Gene

描述（由申请方提供）：神经母细胞瘤（NB）和尤文肉瘤/外周原始神经外胚层肿瘤（ES/PNET）是神经嵴起源的儿科癌症。已经表明，NB的交感神经谱系相对于ES/PNS的副交感神经谱系的分化程序的控制以线性方式进行。NB细胞系和ES/PNET细胞系的体细胞杂交显示NB特异性标志物被抑制，并且用ES/PNET中特有的Ews/FLI-1嵌合基因转染NB显示出类似的结果。本项目将利用蛋白质印迹分析监测p53和pRb的表达，免疫荧光监测p53定位和蛋白质半衰期实验监测p53稳定性来表征Ews/FLI-1在细胞周期控制中的作用。该项目还将利用微芯片阵列技术研究Ews/FLI-1癌基因的机制和途径。

DESCRIPTION (provided by applicant): Neuroblastomas (NB) and Ewing's Sarcoma/peripheral Primitive Neuro-ectodermal Tumors (ES/PNET) are pediatric cancers of neural crest orgin. It has been suggested that the control of the differentiation program o f the sympathetic lineage of NBs versus the parasympathetic lineage of ES/PNETs proceed in a linear fashion. Somatic cell hybridization of NB cell lines and ES/PNET cell lines show that NB-specific markers are repressed, and the transfection of NB with the Ews/FLI-1 chimeric gene characteriztically found in ES/PNETs show similar results. This project will characterize the Ews/FLI-1's role in cell cycle control utilizing western blot analysis to monitor expression of p53 and pRb, immunofluorescence to monitor p53 localization and protein half life experiments to monitor p53 stability. This project will also utilize microchip array techinology to study the mechanism and pathway of the Ews/FLI-1 oncogene.