Fig. 1.

Expression levels of 4′-phosphopantetheinyl proteins in mutant PANK2 fibroblasts. a Immunoblotting analysis of cellular extracts from Control (C) and patients P1, P2 and P3 fibroblasts. Cells were treated with 500 μM pantothenate for 20 days. Protein extracts (50 μg) were separated on a SDS polyacrylamide gel and immunostained with antibodies against PANK1, PANK2, PANK3 and the 4′-phosphopantetheinyl proteins cytosolic FAS and ALD1L1 and mitochondrial mt-ACP, AASS and ALD1L2. b Densitometry of the Western blotting. 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. R = responder fibroblasts