Fig. 1

Effect of pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine treatment on iron accumulation and cell morphology in mutant PANK2 cells with residual PANK2 expression levels. a Control (C1) and PKAN fibroblasts (P1) were treated with pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine at 5 μM for 20 days. Then, cells were stained with Prussian Blue as described in Material and Methods and examined by bright-field microscopy. Scale bar = 15μm. b Quantification of Prussian Blue staining Images were analyzed by the Image J software. c Cell perimeter of untreated and treated Control and PKAN fibroblasts (P1). Images were analyzed by the Image J software. d Total iron content of untreated and treated control and PKAN cells was determined by ICP-MS as described in Material and Methods. Data represent the mean ± SD of three separate experiments. *p < 0.01 between Control and PKAN fibroblasts. ^ap < 0.01 between untreated and treated fibroblasts. A.U., arbitrary units