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Fig. 6 | Orphanet Journal of Rare Diseases

Fig. 6

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

Fig. 6

Effect of α-LA treatment on Fe-S cluster assembly complex proteins and aconitase activity. Control and PKAN fibroblasts (P1, P2, P3) were treated with α-LA at 10 μM for 20 days. a Representative image of NFS1, ISCU and LYRM4 protein levels, proteins involved in Fe-S cluster assembly, analysed by Western blotting of treated and untreated control and PKAN fibroblasts. b Densitometry of Western blotting. c Both mitochondrial and cytosolic aconitase activity were determined by colorimetric assay. Data represent the mean ± SD of three separate experiments. *p < 0.05, **p < 0.005, ***p < 0.005 ****p < 0.0001 between PKAN patients and controls; #p < 0.05, ##p < 0.005, ###p < 0.001 between untreated and treated cells

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