Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Department of Pediatric Hematology and Oncology, Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands.
Mol Oncol. 2018 Feb;12(2):166-179. doi: 10.1002/1878-0261.12149. Epub 2017 Dec 14.
We recently identified hypermethylation at the gene promoter of transcription factor 21 (TCF21) in clear cell sarcoma of the kidney (CCSK), a rare pediatric renal tumor. TCF21 is a transcription factor involved in tubular epithelial development of the kidney and is a candidate tumor suppressor. As there are no in vitro models of CCSK, we employed a well-established clear cell renal cell carcinoma (ccRCC) cell line, 786-O, which also manifests high methylation at the TCF21 promoter, with consequent low TCF21 expression. The tumor suppressor function of TCF21 has not been functionally addressed in ccRCC cells; we aimed to explore the functional potential of TCF21 expression in ccRCC cells in vitro. 786-O clones stably transfected with either pBABE-TCF21-HA construct or pBABE vector alone were functionally analyzed. We found that ectopic expression of TCF21 in 786-O cells results in a trend toward decreased cell proliferation (not significant) and significantly decreased migration compared with mock-transfected 786-O cells. Although the number of colonies established in colony formation assays was not different between 786-O clones, colony size was significantly reduced in 786-O cells expressing TCF21. To investigate whether the changes in migration were due to epithelial-to-mesenchymal transition changes, we interrogated the expression of selected epithelial and mesenchymal markers. Although we observed upregulation of mRNA and protein levels of epithelial marker E-cadherin in clones overexpressing TCF21, this did not result in surface expression of E-cadherin as measured by fluorescence-activated cell sorting and immunofluorescence. Furthermore, mRNA expression of the mesenchymal markers vimentin (VIM) and SNAI1 was not significantly decreased in TCF21-expressing 786-O cells, while protein levels of VIM were markedly decreased. We conclude that re-expression of TCF21 in renal cancer cells that have silenced their endogenous TCF21 locus through hypermethylation results in reduced clonogenic proliferation, reduced migration, and reduced mesenchymal-like characteristics, suggesting a tumor suppressor function for transcription factor 21.
我们最近在肾透明细胞肉瘤(CCSK)中鉴定出转录因子 21(TCF21)基因启动子的超甲基化,这是一种罕见的小儿肾肿瘤。TCF21 是一种参与肾脏管状上皮发育的转录因子,是候选肿瘤抑制因子。由于没有 CCSK 的体外模型,我们使用了一种成熟的肾透明细胞癌(ccRCC)细胞系 786-O,该细胞系的 TCF21 启动子也表现出高甲基化,导致 TCF21 表达水平降低。TCF21 的肿瘤抑制功能尚未在 ccRCC 细胞中得到功能验证;我们旨在探索 TCF21 在 ccRCC 细胞中的体外功能潜力。我们对稳定转染 pBABE-TCF21-HA 构建体或单独 pBABE 载体的 786-O 克隆进行了功能分析。我们发现,在 786-O 细胞中外源性表达 TCF21 导致细胞增殖呈下降趋势(无显著差异),与 mock 转染的 786-O 细胞相比,迁移明显减少。虽然集落形成实验中 786-O 克隆形成的菌落数量没有差异,但表达 TCF21 的 786-O 细胞中的菌落大小明显减小。为了研究迁移的变化是否是由于上皮-间充质转化的变化,我们检测了选定的上皮和间充质标志物的表达。虽然我们观察到在过表达 TCF21 的克隆中上皮标志物 E-钙黏蛋白的 mRNA 和蛋白水平上调,但这并没有导致通过荧光激活细胞分选和免疫荧光测量的 E-钙黏蛋白表面表达。此外,TCF21 表达的 786-O 细胞中间充质标志物波形蛋白(VIM)和 SNAI1 的 mRNA 表达没有显著降低,而 VIM 的蛋白水平明显降低。我们得出的结论是,在通过超甲基化沉默其内源性 TCF21 基因座的肾癌细胞中重新表达 TCF21 会导致克隆形成增殖减少、迁移减少和间充质样特征减少,提示转录因子 21 具有肿瘤抑制功能。