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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

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

Fig. 5

Effect of α-LA on mitochondrial complex I. Control and PKAN fibroblasts (P1, P2, P3) were treated with α-LA at 10 μM for twenty days. a MT-ND1 and NDUFA9, mitochondrial complex I subunits, analysed by Western blotting. b Densitometry of Western blotting. c Mitochondrial complex I activity in whole cellular extracts was measured as described in Material and Methods. d Quantification of mitochondrial complex I activity. Data represent the mean ± SD of two separate experiments. **p < 0.005, ***p < 0.001 ****p < 0.0001 between PKAN patients and controls; ##p < 0.005, ###p < 0.001 ####p < 0.0001 between untreated and treated cells

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172