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Fig. 4 | Orphanet Journal of Rare Diseases

Fig. 4

From: Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives

Fig. 4

Expression levels of lipoyl proteins, Fe-S cluster complex proteins and mitochondrial complex I subunits in mutant PANK2 fibroblasts. Control (C) and PKAN fibroblasts (P1 and P3) were treated with 500 μM pantothenate for 20 days. a Immunoblotting analysis of cellular extracts from Control (C) and patients P1, P2 and P3 fibroblasts. Protein extracts (50 μg) were separated on a SDS polyacrylamide gel and immunostained with antibodies against lipoic acid, Fe-S cluster complex proteins NFS1, ISD11 and LYRM4 and mitochondrial complex I subunits MT-ND1 and NDUFA9. 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 cells. A.U., arbitrary units. R = responder fibroblasts

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172