Hoffman L M, Brooks S E, Amsterdam D, Oropello J, Schneck L
J Neurosci Res. 1980;5(5):413-7. doi: 10.1002/jnr.490050506.
A human Tay-Sachs disease (TSD) fetal-brain-cell line is a useful model for the disease since the cells lack hexosaminidase A and accumulate the ganglioside, GM2. This brain-cell line was used to assess the effect of hexosaminidase A treatment on GM2 storage material. Entry of placental hexosaminidase A into the cells was obtained by pretreatment of the cultures with concanavalin A. Cells were analyzed periodically during six days. During the course of the experiment, GM2 in the cells decreased by approximately 50%. A substantial amount of hexosaminidase A was maintained in the cultures throughuot the experiment. This strategy was successful in mobilizing stored GM2 in TSD brain-cell cultures. Therefore, the activating factor needed for hexosaminidas A activity must be present in TSD-cultured brain cells.
人类泰-萨克斯病（TSD）胎儿脑细胞系是研究该疾病的有用模型，因为这些细胞缺乏己糖胺酶A并积累神经节苷脂GM2。该脑细胞系用于评估己糖胺酶A治疗对GM2储存物质的影响。通过用伴刀豆球蛋白A预处理培养物，使胎盘己糖胺酶A进入细胞。在六天内定期对细胞进行分析。在实验过程中，细胞中的GM2减少了约50%。在整个实验过程中，培养物中一直维持着大量的己糖胺酶A。该策略成功地动员了TSD脑细胞培养物中储存的GM2。因此，己糖胺酶A活性所需的激活因子必定存在于TSD培养的脑细胞中。
