Fig. 1

Region-biased depletion of CD11b+ and Sn+ cells in the CNS of CLN1 mice upon treatment with PLX3397. a, b Representative fluorescent microscopic images of immunohistochemically labeled CD11b⁺ or activated Sn⁺ microglia/macrophages in longitudinal optic nerve sections of 6-month-old Ppt1^+/+, Ppt1^−/−, and PLX3397-treated Ppt1^−/− mice after 5 months of treatment. Scale bar: 50 μm. c, d Quantification of CD11b⁺ or Sn⁺ cells showed a significant reduction in number in optic nerves after PLX3397 treatment. e, f Representative fluorescent microscopic images of immunohistochemically labeled CD11b⁺ or activated Sn⁺ microglia/macrophages in S1Bf cortex region of 6-month-old Ppt1^+/+, Ppt1^−/− mice, and PLX3397-treated Ppt1^−/− mice after 5 months of treatment. Scale bar: 50 μm. g, h Quantification of CD11b⁺ or Sn⁺ cells showed a significant or non-significant tendential reduction in number in the S1Bf cortex region after PLX3397 treatment, respectively. Percentages of Sn⁺ cells related to CD11b⁺ cells are indicated below the corresponding groups. n = 5 male and 5 female mice per group. One-way ANOVA and Tukey’s post hoc tests. *P < 0.05; ***P < 0.001