Fig. 4

Neuropathological analysis of brain tissue from MJD transgenic mice treated with 2% Trehalose. Cresyl violet staining was performed on left hemicerebellum sections from MJD transgenic female mice treated with distilled water (control; n = 7) or 2% Trehalose (n = 7) for 30 weeks. a Representative picture of cerebellum layers from control and 2% Trehalose-treated MJD transgenic mice. Abbreviations: GL, Granular layer; ML, Molecular layer; PCL, Purkinje cell layer. Scale = 50 µm. b, c Trehalose has no effect in cerebellum volume, but increased the thickness of cerebellum layers compared to controls. Data are presented as mean ± SEM. Statistical analysis was performed using the two-tailed Student’s t test. * < 0.05, comparing to controls; ns = not significant. Immunofluorescent staining was also performed on left hemicerebellum sections from MJD transgenic mice treated with distilled water (control; n = 7) or 2% Trehalose (n = 7) for 30 weeks. d Representative confocal image of immunofluorescent staining against HA (MutAtx-3; green) and calbindin (red) of left hemicerebellum sections from MJD transgenic mice treated with distilled water (control; n = 7) or 2% Trehalose (n = 7) for 30 weeks. Scale bar = 10 µm. e Representative picture of aggregates size from control and 2% Trehalose-treated MJD transgenic mice. Scale bar = 5 µm. f, g The total number of aggregates and aggregates size (diameter) in a representative lobule (lobule IX) were quantified. Trehalose had no effect in total number of aggregates, but decreased aggregates diameter in lobule IX. Data are presented as mean ± SEM. Statistical analysis was performed using the two-tailed Student’s t test. ns not significant