Hoffman L M, Amsterdam D, Schneck L
Brain Res. 1976 Jul 23;111(1):109-17. doi: 10.1016/0006-8993(76)91052-0.
Cultured cells derived from Tay-Sachs disease (TSD) fetal cerebellum were shown to accumulate GM2 ganglioside when compared with control cultures. In contrast, fibroblasts derived from TSD fetal lung do not contain GM2. GM2 was identified by thin-layer chromatography (TLC) and confirmed by gas-liquid chromatography (GLC). Unlike fetal TSD brain, the cell cultures established from fetal TSD brain have high concentrations of globoside and GD3 and little or no asialo GM2(GA2). The GM2 and related glycosphingolipids in these cultured cells contain a high percentage of C24:0 and C24:1 fatty acids. In spite of these differences, this TSD cell strain is unique in that it accumulates GM2, and can therefore serve as a useful in vitro model for the study of TSD.
与对照培养物相比，来自泰-萨克斯病（TSD）胎儿小脑的培养细胞显示出会积累GM2神经节苷脂。相比之下，来自TSD胎儿肺的成纤维细胞不含GM2。GM2通过薄层色谱法（TLC）鉴定，并通过气液色谱法（GLC）确认。与胎儿TSD脑不同，从胎儿TSD脑建立的细胞培养物中含有高浓度的球苷脂和GD3，几乎没有或没有脱唾液酸GM2（GA2）。这些培养细胞中的GM2和相关糖鞘脂含有高比例的C24:0和C24:1脂肪酸。尽管存在这些差异，但这种TSD细胞系的独特之处在于它会积累GM2，因此可作为研究TSD的有用体外模型。
