Fig. 8

Effect of pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine treatment on PANK 2 and mtACP expression levels in mutant PANK2 cells with residual PANK2 expression levels and mutant cells with expression of truncated PANK2. PKAN fibroblasts were treated with pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine at 5 μM for 20 days. Protein extracts (50 μg) were separated on a SDS polyacrylamide gel and immunostained with antibodies against PANK2 and mtACP. Actin was used as a loading control. a Western blotting of mutant of P2 fibroblasts. b Densitometry of Western blotting of mutant P2 fibroblasts. c Western blotting of mutant of P3 fibroblasts. d Densitometry of Western blotting of mutant P3 fibroblasts. e Western blotting of mutant of P4 fibroblasts. f Densitometry of Western blotting of mutant P4 fibroblasts. g Western blotting of mutant of P5 fibroblasts. h Densitometry of Western blotting of mutant P5 fibroblasts. Data represent the mean ± SD of three separate experiments. *p < 0.01 between PKAN patients and controls. ^ap < 0.01 between untreated and treated fibroblasts. A.U., arbitrary units