Fig. 9
Scavenging mitochondrial oxidative stress and inhibiting lipid peroxidation rescues proteinuria in DBP-EGFP cystinosis zebrafish larvae. a. Quantification of DBP-EGFP fluorescence intensity in the eyes of 120 h post-fertilization (hpf) zebrafish larvae. Data include WT (ctns+/+), ctns−/−, and ctns−/− larvae treated from 48 to 120 hpf with 100 µM cysteamine, 100 µM cysteamine + 10 µM MitoTEMPO, 10 µM liproxstatin-1, or 100 µM cysteamine + 10 µM liproxstatin-1. n ≥ 60 zebrafish larvae per condition. Statistical analysis: Šídák's One-Way ANOVA. Fluorescence intensity is normalized to WT and presented as a violin plot. b. Representative images of 120 hpf zebrafish larvae eyes for WT, ctns−/−, and ctns−/− treated with 100 µM cysteamine, 10 µM MitoTEMPO, 100 µM cysteamine + 10 µM MitoTEMPO, 10 µM liproxstatin-1, or 100 µM cysteamine + 10 µM liproxstatin-1. Red circles highlight areas where DBP-EGFP fluorescence intensity was measured. Scale bar: 250 µm |