Fig. 4

Effect of pantothenate, pantethine, omega-3 and vitamin E supplementation on PANK2 and mtACP expression levels. (A) Expression levels of PANK2 and mtACP in Control (C1 and C2) and PKAN cells (P1, P2 and P3) assessed by Western blotting. (B) Quantification of PANK2 and mtACP expression levels. (C) Controls (C1) and patient P1 fibroblasts were treated with 5 µM pantothenate, 5 µM pantethine, 5 µM vitamin E and 5 µM omega 3 for 10 days (+ T). PANK2 and mtACP protein expression levels were analysed by Western blotting. Tubulin was used as loading control. (D) Densitometry of the Western blotting of PANK2 and mtACP under pantothenate, pantethine, omega-3 and vitamin E supplementation. (E) PANK2 transcripts were quantified by RT-qPCR. Data represent the mean ± SD of three separate experiments. *p < 0.01 between PKAN patients and controls. ^ap < 0.05 and ^aap < 0.01 between untreated and treated fibroblasts. A.U., arbitrary units. Unedited and uncut blots are shown in Supplementary Figs. 1 and 2