FLX reduces lysosomal vacuolization and induces exocytosis in MPS-IIIA liver tissues
(A) Representative confocal images of LAMP-1 in liver sections from WT (n = 4 per groups) and MPS-IIIA (n = 5 per groups) mice that were FLX-treated and compared with the vehicle-treated groups. The plot shows the mean intensity of LAMP-1 per area ±SEMs. ANOVA test, ∗∗∗p < 0.001 versus WT; ⋅⋅⋅p < 0.001 versus MPS-IIIA vehicle. (B) Representative EM images of liver tissues from WT and MPS-IIIA mice vehicle- or FLX-treated. The plot shows the diameter (nm) of n > 50 lysosomal-like structures derived from 2 different animals per group. ANOVA test, ∗∗∗p < 0.001 versus WT; ⋅⋅⋅p < 0.001 versus MPS-IIIA. The 2 histograms analyze the size-frequency distribution. The black arrows indicate the vesicle structures. (C) Representative EM images of liver tissues from vehicle- and FLX-treated MPS-IIIA mice showing the distance of the vesicles to the PM. The plot shows the number of vesicles close to the PM as ±SEMs of n > 50 lysosomal-like structures derived from 2 different animals per group. ANOVA test, ∗∗∗p < 0.001 versus vehicle. The black arrows indicate the vesicle structures; the white arrowheads indicate the PM. BC, bile canaliculus.