Figure 7.
Ambroxol Treatment Upregulates Macroautophagy and Rescues the Autophagic Defect
(A–D) Following ambroxol (ABX) treatment, levels of the autophagosome markers LC3-II and p62 were significantly increased in GBA1 mutation (Het1–3; N370S/wt) and iPD (iPD1–3) cell lines, but not in control (C1–3; CTRL) cells.
(E) LC3B mRNA levels were not significantly different between treated and untreated groups.
(F) p62 mRNA levels were increased in iPD neurons after ambroxol treatment, but the increase was not statistically significant in control and GBA1 mutation cells.
(G–J) When the macroautophagy pathway was inhibited with bafilomycin in the ambroxol-treated neurons, LC3-II and p62 levels were increased further, indicating that the fusion of lysosomes with autophagosomes is normal but the autophagosome number is increased by the ambroxol treatment in GBA1 mutation and iPD groups.
For all three groups n = 3 cell lines; data are presented as means ± SEM. All comparisons were carried out with one-way ANOVA and Student's t test. ∗∗p < 0.01; ∗p < 0.05; NS, not significant (p > 0.05).