Figure 2.

Fingolimod and Teriflunomide Prevent the Elevation of CD8⁺ T Lymphocyte Numbers in CLN1 and CLN3 Disease Models

(A) Representative immune fluorescence microscopy of CD8⁺ T lymphocytes (arrows) in longitudinal optic nerve sections from 6-month-old Ppt1^+/+, Ppt1^−/−, fingolimod-treated Ppt1^−/− (+ FTY720), and teriflunomide-treated Ppt1^−/− (+ Teriflunomide) mice (top) and 17-month-old Cln3^+/+, Cln3^−/−, fingolimod-treated Cln3^−/− (+ FTY720), and teriflunomide-treated Cln3^−/− (+ Teriflunomide) mice (bottom). Scale bar, 20 μm. (B) Quantification of CD8⁺ T lymphocytes in optic nerve sections. The numbers of CD8⁺ T cells were significantly increased in the untreated CLN1 and CLN3 disease models compared with WT mice. This increase was prevented in both models by treatment with fingolimod as well as with teriflunomide for 150 days (n = 5 mice/group). One-way ANOVA and Tukey’s post hoc tests. ***p < 0.001. (C) Representative light microscopy of CD8⁺ T lymphocytes (circles) in coronal sections of S1BF cortex from 6-month-old Ppt1^+/+, Ppt1^−/−, fingolimod-treated Ppt1^−/− (+ FTY720), and teriflunomide-treated Ppt1^−/− (+ Teriflunomide) mice (top) and 17-month-old Cln3^+/+, Cln3^−/−, fingolimod-treated Cln3^−/− (+ FTY720), and teriflunomide-treated Cln3^−/− (+ Teriflunomide) mice (bottom). Note the reduction of cortical atrophy in treated Ppt1^−/− mice. Scale bar, 50 μm. (D) Higher magnification of CD8⁺ T lymphocytes. Scale bar, 20 μm.