Fig. 2

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. a Control (C1) and PKAN fibroblasts (P1) were treated with pantothenate (Pant), pantethine (Pantethi), vitamin E (Vit E), omega 3 (O3), L-carnitine (L-carnit) or thiamine (Tiam) 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. b Densitometry of the Western blotting of PANK2. c Densitometry of the Western blotting of mtACP. 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