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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

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

Fig. 5

Reduced PDH, aconitase activities and complex I activity in mutant PANK2 fibroblasts. Control (C), P1 and P3 fibroblasts were treated with 500 μM pantothenate for 20 days. a, b PDH activity in whole cellular extracts was determined as described in Material and Methods. c, d Cytosolic and mitochondrial aconitase activity in whole cellular extracts was determined as described in Material and Methods. e, f Mitochondrial complex I activity in whole cellular extracts was determined as described in Material and Methods. 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