Figure 2 | Scientific Reports

Figure 2

From: Pre-diagnosing and managing patients with GM1 gangliosidosis and related disorders by the evaluation of GM1 ganglioside content

Figure 2

Miglustat reduces the amount of GM1 ganglioside in fibroblasts from GM1 gangliosidosis patients. (A) Primary cultures of fibroblasts isolated from patients with different degrees of disease severity were incubated for 5 days with different concentrations of Miglustat and subsequently fixed, permeabilized and labelled with biotinylated CTXb - streptavidin Alexa_488. The mean fluorescence intensity per cell decreases with increasing concentrations of Miglustat, both in infantile and juvenile patients. At the bottom, representative images of the decrease for control, juvenile and infantile patients. Scale bar 20 µm > 20 cells were analysed. Error bar S.D. ANOVA test ***P < 0.001. (B) Primary cultures of fibroblasts isolated from control and infantile patient were incubated for different lengths of time with 1 mM Miglustat and subsequently fixed, permeabilized and labelled with biotinylated CTXb - streptavidin Alexa_488 > 20 cells were analysed. Error bar S.D. Student’s t test *P < 0.05, ***P < 0.001.

Back to article page