The biological and clinical heterogeneity of neuroblastoma is closely associated with signaling pathways that control cellular characteristics such as proliferation, survival and differentiation. The Shc family of docking proteins is important in these pathways by mediating cellular signaling. In this study, we analysed the expression levels of ShcA and ShcC proteins in 46 neuroblastoma samples and showed that a significantly higher level of ShcC protein is observed in neuroblastomas with poor prognostic factors such as advanced stage and MYCN amplification (P<0.005), whereas the expression level of ShcA showed no significant association with these factors. Using TNB1 cells that express a high level of ShcC protein, it was demonstrated that knockdown of ShcC by RNAi caused elevation in the phosphorylation of ShcA, which resulted in sustained extracellular signal-regulated kinase activation and neurite outgrowth. The neurites induced by ShcC knockdown expressed several markers of neuronal differentiation suggesting that the expression of ShcC potentially has a function in inhibiting the differentiation of neuroblastoma cells. In addition, marked suppression of in vivo tumorigenicity of TNB1 cells in nude mice was observed by stable knockdown of ShcC protein. These findings indicate that ShcC is a therapeutic target that might induce differentiation in the aggressive type of neuroblastomas.

译文

:神经母细胞瘤的生物学和临床异质性与控制细胞特征(例如增殖,存活和分化)的信号通路密切相关。 Shc家族的对接蛋白通过介导细胞信号传导在这些途径中很重要。在这项研究中,我们分析了46个神经母细胞瘤样品中ShcA和ShcC蛋白的表达水平,并发现在预后较差的神经母细胞瘤(如晚期和MYCN扩增)中观察到ShcC蛋白水平显着升高(P <0.005),而ShcA的表达水平与这些因素无显着相关性。使用表达高水平ShcC蛋白的TNB1细胞,已证明RNAi抑制ShcC会导致ShcA磷酸化水平升高,从而导致持续的细胞外信号调节激酶激活和神经突生长。 ShcC敲低诱导的神经突表达了几种神经元分化标记,这表明ShcC的表达可能具有抑制神经母细胞瘤细胞分化的功能。另外,通过稳定敲除ShcC蛋白观察到裸鼠体内TNB1细胞体内致瘤性的显着抑制。这些发现表明,ShcC是一种治疗靶标,可能在侵袭性类型的神经母细胞瘤中诱导分化。

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