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Fig. 7 | Orphanet Journal of Rare Diseases

Fig. 7

From: Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

Fig. 7

Effect of pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine treatment on lipid peroxidation, PDH activity and mitochondrial complex I activity in mutant PANK2 cells with residual PANK2 expression levels. P1 fibroblasts were treated with pantothenate, pantethine, vitamin E, omega 3, L-carnitine or thiamine at 5 μM for 20 days. a Lipid peroxidation was assessed by BODIPY staining and fluorescence microscopy analysis. b Quantification of BODIPY signal. c PDH activity in whole cellular extracts was determined as described in Material and Methods. d Quantification of PDH activity. e Mitochondrial complex I activity in whole cellular extracts was determined as described in Material and Methods. f Quantification of Complex I activity Data represent the mean ± SD of three separate experiments. *p < 0.01 between PKAN patients and controls; ap < 0.01 between untreated and treated cells

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172