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Figure 3
Delayed treatment with BLD-2736 or calpeptin produced a therapeutic benefit, despite administration after the onset of disease pathology. (A) Timeline displaying the onset of disease pathology at 2 dpf, treatment administration at 4 dpf, and examination of treatment efficacy at 6 dpf. (B) Treatment of SCA3 zebrafish from 4 to 6 dpf improved the distance swum when compared to vehicle treated SCA3 zebrafish. Swimming analysis was performed on n = 12–25 larvae per group. (C) Post-symptomatic treatment with calpain inhibitors produced a detectable decrease in the presence of detergent-insoluble EGFP-fused ataxin-3 particles. Each data point represents an experimental replicate, where n = 12–20 larvae were pooled together. All data are presented as mean ± SEM. * represents p < 0.05, ** represents p < 0.01, **** represents p < 0.0001.