Fig. 4.

p32-inhibitor treatment ameliorates the phenotypes of mutant hCHCHD2 transgenic Drosophila. A Representative confocal images of the 40-day-old adult Drosophila brains stained for TH expression (*) in the PPL1 + PPL2c and PPL2ab clusters, and quantitation. For each group, a total of 6 to 20 brain samples were analyzed (n = 3, One-way ANOVA with Bonferroni post hoc test). B Climbing ability was monitored in adult Drosophila aged to 45 days and 55 days using negative geotaxis assay. For each group, approximately 120 to 150 flies were examined at day 45 of aging (n = 5 for all groups, except n = 4 for Arg145Gln group). Each experimental repeat was accompanied by three technical repeats. Statistics used is Pearson Chi-Square (df = 1). C Measurement of H₂O₂ levels in the transgenic Drosophila expressing hCHCHD2, with or without p32-I treatment (n = 4 for all groups, except n = 6 for p32-I – WT group and n = 8 for WT group). Statistics used is One-way ANOVA with Bonferroni post hoc test. D Kaplan–Meier analysis of survival rates in the transgenic Drosophila expressing hCHCHD2, with or without p32-I treatment. For each group, the total numbers of flies scored are between 158 and 276 (n = 3). The concentration of p32-I used is 25 µM. n = experimental repeats. Data are presented as mean ± SEM. ^*p < 0.05; ^**p < 0.01