摘要目的 探讨转录因子Sp1调节儿童横纹肌肉瘤成纤维细胞生长因子受体4(FGFR4)基因表达的作用及其调节的分子机制.方法 应用细胞及分子生物学技术如细胞转染、RNA和蛋白质印迹等方法检测2种不同横纹肌肉瘤细胞株中的FGFR4 mRNA和蛋白的表达,观察Sp1对FG-FR4表达的调节作用,探讨Sp1凋节FGFR4基因表达的机制.结果 Sp1在儿童横纹肌肉瘤中可高度表达,Sp1与FGFR4启动区结合并上调了FGFR4的表达.Sp1的这一调节作用不受FGFR4启动区甲基化作用的影响.结论 Sp1促进FGFR4在儿童横纹肌肉瘤细胞中的表达,该作用可能是通过Sp1与FGFR4启动区的顺式元件直接结合而实现的.

abstractsObjective To investigate the role of transcription factor Sp1 in the fibroblast growth factor receptor 4 (FGFR4) expression regulation and to obtain insight into the regulatory mechanisms goveming FGFR4 gene regulation and overexpression in human infant rhabdomyosarcomas. Methods By using a series of cellular and molecular biology methods like transfection, Northern blotting, Western blotting etc., the mRNA and protein expression of FGFR4 was detected. The regulatory effects of Sp1 on the FGFR4 expression in the human rhabdomyosarcoma-derived cells were analyzed. Results Sp1 could be express highly in the human rhabdomyosarcoma-derived cells, and its binding to FGFR4 promoter could upregulate the FGFR4 expression, which was influenced by the methylation of FGFR4 promoter. Conclusion Sp1 induces the expression of FGFR4 in human infant rhabdomyosarcomas by directly binding of Sp1 with DNA cis elements in FGFR4 gene promoter region.