Goji J, Sano K, Nakamura H, Ito H
Department of Pediatrics, Kobe University School of Medicine, Japan.
Cancer Res. 1992 Aug 1;52(15):4214-20.
We have established a cell line (KU-SN) from a peripheral neuroectodermal tumor originating in the left scapula of a 4-year-old girl. The original tumor was immunoreactive with antibodies for neurofilament proteins, neuron-specific enolase, vimentin, S100 protein, and beta 2-microglobulin. Dense core granules, 50-150 nm in diameter, were identified by electron microscopy. The cell line was established from tumor cells in metastatic lung fluid. KU-SN cells were immunoreactive with the antibodies for neurofilament proteins, vimentin, neuron-specific enolase, S100 protein, glial fibrillary acidic protein, cytokeratin, and carcinoembryonic antigen. Besides these neuronal features, KU-SN cells express type 2 collagen and insulin-like growth factor 1 receptor. The addition of insulin-like growth factor 1 (100 ng/ml) increased the growth rate of KU-SN cells 2.1-fold over control. Some cells were positive for Alcian blue and alkaline phosphatase staining. Cytogenetic analysis of KU-SN cells disclosed a reciprocal chromosomal translocation [t(11,22)]. Northern blot analysis of KU-SN cells demonstrated amplified expression of the c-myc gene but not the N-myc gene. When tumor cells were transplanted into nude mice, cartilage was formed. The cartilage was immunoreactive with the antibody for HLA-ABC, indicating that it was derived from the tumor cells, not from mouse tissue. Chondrocytic differentiation was not observed in xenografts of Ewing's sarcoma cell lines SK-ES or RD-ES or the peripheral neuroectodermal tumor cell line SK-N-MC. These results indicate that KU-SN cells represent primitive neural crest cells having the potential for chondrocytic differentiation.
我们从一名4岁女童左肩胛骨的外周神经外胚层肿瘤中建立了一个细胞系（KU-SN）。原始肿瘤对神经丝蛋白、神经元特异性烯醇化酶、波形蛋白、S100蛋白和β2-微球蛋白的抗体呈免疫反应。通过电子显微镜鉴定出直径为50-150nm的致密核心颗粒。该细胞系是从转移性肺液中的肿瘤细胞建立的。KU-SN细胞对神经丝蛋白、波形蛋白、神经元特异性烯醇化酶、S100蛋白、胶质纤维酸性蛋白、细胞角蛋白和癌胚抗原的抗体呈免疫反应。除了这些神经元特征外，KU-SN细胞还表达2型胶原蛋白和胰岛素样生长因子1受体。添加胰岛素样生长因子1（100ng/ml）使KU-SN细胞的生长速率比对照增加了2.1倍。一些细胞对阿尔辛蓝和碱性磷酸酶染色呈阳性。KU-SN细胞的细胞遗传学分析显示出相互染色体易位[t(11,22)]。KU-SN细胞的Northern印迹分析表明c-myc基因表达扩增，但N-myc基因未扩增。当将肿瘤细胞移植到裸鼠中时，形成了软骨。该软骨对HLA-ABC抗体呈免疫反应，表明它源自肿瘤细胞，而非小鼠组织。在尤因肉瘤细胞系SK-ES或RD-ES或外周神经外胚层肿瘤细胞系SK-N-MC的异种移植中未观察到软骨细胞分化。这些结果表明KU-SN细胞代表具有软骨细胞分化潜能的原始神经嵴细胞。
