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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Alpha-lipoic acid supplementation corrects pathological alterations in cellular models of pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

Fig. 1

Effect of α-LA supplementation on iron accumulation in three mutant PANK2 cells. a Control (C1) and three PKAN fibroblast cell lines (P1, P2 and P3) were treated with increasing α-LA concentrations (1, 10, 50, 100 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 = 15 μm. Quantification of Prussian Blue staining is shown in Additional file 1: Fig. 1 b Iron accumulation determined by colorimetric Ferrozine-based assay. Significance between PKAN and control fibroblasts is represented as ****p < 0.0001, **p < 0.005, *p < 0.05 fibroblasts and ####p < 0.0001, #p < 0.05 between untreated and treated fibroblasts

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172