Fig. 11.

Effect of α-LA treatment on lipofuscin accumulation. Control and PKAN fibroblasts (P1, P2, P3) were treated with α-LA at 10 μM for 20 days. a Representative images of lipofuscin staining by SBB of untreated and treated control and three PKAN patient fibroblasts. Scale bar = 20 μm. b SBB staining quantification. Data represent the mean ± SD of three separate experiments (50 cell images for each condition). *p < 0.05, **p < 0.05, ****p < 0.001 between PKAN patients and controls. ^#p < 0.05 between untreated and treated fibroblasts. A.U., arbitrary units