Figure 1

Behavioral and morphological analysis of Elavl3^−/− mice. (A–C) Behavioral analysis of Elavl3^−/− mice. (A) Representative forelimb footprints of 7-months-old Elavl3^+/+ and Elavl3^−/− mice. (B) Aged (6–7 months old) Elavl3^−/− mice exhibited significantly wider forelimb base width compared to Elavl3^+/+ mice (n = 7, 7, 6, and 7 mice for each group). (C) Aged Elavl3^−/− mice exhibited tremor during walking (n = 7, 7, 6, and 7 mice for each group). These phenotypes were not detected in young adult (2–3 months old) mice. **P < 0.01, ***P < 0.001, NS, not significant (Student’s t-test). (D and E) The cerebellar sections of Elavl3^+/+ and Elavl3^−/− mice at 3, 6, 9, and 14 months of age were immunostained for calbindin and MAP2. (D) Axons of Elavl3^−/− Purkinje cells were swollen (spheroid formation) in the granule cell layer. Arrows indicate spheroids. Scale bar, 20 μm. (E) Axonal terminals of Elavl3^−/− Purkinje cells were swollen and synaptic formation was disrupted in the cerebellar nuclei with age. The density of axonal terminals of Purkinje cells at the cerebellar nuclei is quantified in Supplementary Fig. S2F and G. Arrows and asterisks indicate swollen axonal terminals and somata of cerebellar nuclei neurons, respectively. Scale bar, 10 μm. (F) Schematic model showing the degenerative processes of axons of Elavl3^−/− Purkinje cells.