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. 2014 Sep 26;24(3):727–739. doi: 10.1093/hmg/ddu491

Figure 2.

Figure 2.

Progressive Purkinje cell loss and axonal swellings in Sacs−/− are restricted to the anterior lobules. (A–C) Anti-calbindin D-28K immunohistochemistry on mid-sagittal brain sections from 365-day-old Sacs+/+ (A) and Sacs−/− (B) mice revealed Purkinje cell loss in the cerebellar anterior lobules, II to V of Sacs−/− mice (arrows). On coronal sections of Sacs−/− brain, Purkinje cell loss was restricted to the vermis and the medial hemispheres, namely the simple (SIM) and ansiform crus 1 (ANcr1) lobules, with sparing of the parafloccular lobe (PFL in C). (D and E) Neuronal cell counts in the anterior (I to VI) and posterior (VII to X) lobules at different ages, showing significant loss of Purkinje cells in Sacs−/− mice starting at 90 days of age in the anterior lobules (D), but not in the posterior lobules (E). Data represent means ± SD, n = 4–5 mice per group. (F–I) Anti-calbindin D-28K labeling of cerebellar parasagittal brain sections of 30-day-old Sacs−/− mice revealing dystrophic axons (asterisk and arrowhead in G) and occasional torpedo swellings in the proximal axonal segment (arrow in G) compared with Sacs+/+ mice (F) (scale bar = 40 µm). (I) Numerous axonal swellings (arrows) and beaded axons (asterisk) were present in Sacs−/− samples at 90 days of age compared with age-matched controls (H) (scale bar = 100 µm). (J) Significant increase in numbers of axonal swellings in the cerebellar anterior lobules of Sacs−/− mice. Data represent means ± SD, n = 3 mice per group; *P < 0.05, **P < 0.01, ***P < 0.001 (unpaired t-test).