Skip to main content
. 2010 Jun 22;18(8):1519–1526. doi: 10.1038/mt.2010.113

Figure 4.

Figure 4

Enzyme replacement effect of NgHexA on reduction of intracellular GM2 gangliosides. Reduction of accumulated GM2 was quantified by means of Cell ELISA and immunofluoroscense with anti-GM2 antibodies. (a) Quantitative analysis of accumulated GM2. WT- or NgHexA was administered to SD fibroblasts, followed by incubation for 3 days, and then the GM2 level was determined by Cell ELISA. In another experiment, HexA [4-methylumbelliferyl N-acetyl-β--glucosaminide (4-MUG) activity, 150 nmol/hour] was administered after treatment with 10 mmol/l M6P for 30 minutes. Each error bar represents the average of three or four experiments, and shows ±SEM, ***P < 0.005 (Tukey's post hoc test). (b) Immunofluorescense analysis. WT- or NgHexA (4-MUG activity, 500 nmol/hour) was administered to SD fibroblasts, followed by incubation for 3 days, accumulated GM2 being visualized as immunofluororescense with anti-GM2. (A) Untreated; (B) treated with WTHexA (4-MUG activity, 800 nmol/hour/well); (C) treated with NgHexA (800 nmol/hour/well); (D) untreated WT fibroblasts; (E) treated with 5 mmol/l M6P/WTHexA (800 nmol/hour/well) after treatment with 5 mmol/l M6P; and (F) treated with NgHexA (800 nmol/hour/well) after treatment with 5 mmol/l M6P. Bar = 50 µm.