Fig. 7

Depletion of microglia/macrophages improves clinical outcome in CLN1 mice. a Longitudinal analysis of cumulative rotarod performance from 4 to 6 months of age in Ppt1^+/+, Ppt1^−/−, and PLX3397 treated Ppt1^−/− mice. Motor impairment was attenuated in PLX3397 treated Ppt1^−/− mice. b Visual acuity measured by optokinetic responses showed that PLX3397-treatment significantly improved visual acuity in Ppt1^−/− mice. c Kaplan-Meier survival analysis demonstrated increased longevity of CLN1 mice upon treatment. Log-rank test. d, e Quantification of myoclonic jerks in 6-month-old Ppt1^+/+, Ppt1^−/−, and PLX3397-treated Ppt1^−/− mice showed that their frequency in Ppt1^−/− mice was significantly reduced upon PLX3397 treatment. This treatment effect was more pronounced in male mutants compared to their female counterparts. n = 5 male and 5 female mice per group. One-way ANOVA and Tukey’s post hoc tests. *^{, #}P < 0.05; **P < 0.01; ***P < 0.001. # significant difference to untreated Ppt1^−/− mice of the same sex