Fig. 8: Calpain inhibition in Kctd7^–/– mice ameliorates behavioral impairments and neurodegeneration.

a Schematic diagram of drug treatment, tissue preparation and behavioral tests. b, c Immunoblot analysis of cerebellar tissue from 8-week-old WT and Kctd7^–/– mice treated with E-64 or vehicle using α-spectrin antibody, n = 3 for WT (untreated and treated), n = 4 for Kctd7^–/– (untreated and treated). d–f Confocal microscopy analysis of the cerebellum from 8-week-old WT and Kctd7^–/– mice treated with E-64 or vehicle. α-calbindin antibody and α-GFAP antibody were used to label Purkinje cells (d) and astrocytes (f), respectively. Scale bars, 200 μm. Quantitation of Purkinje cell numbers is reported in e, n = 5. g, h Immunoblot analysis of cerebellar tissue from 8-week-old WT and Kctd7^–/– mice treated with E-64 or vehicle using α-GFAP antibody, n = 3 for WT (untreated and treated), n = 4 for Kctd7^–/– (untreated and treated). i Rotarod test measuring the latency to fall for 8-week-old WT and Kctd7^–/– mice treated with E-64 or vehicle. n = 10 for vehicle-treated mice (WT and Kctd7^–/–). n = 12 for E-64-treated mice (WT and Kctd7^–/–). j Inverted pole test measuring the time used to climb down from the pole for 8-week-old WT and Kctd7^–/– mice treated with E-64 or vehicle. n = 10 for vehicle-treated mice (WT and Kctd7^–/–). n = 12 for E-64-treated mice (WT and Kctd7^–/–); ^∗P < 0.05, ^∗∗P < 0.01, ^∗∗∗P < 0.001, ns not significant.