Skip to main content
Fig. 7 | Orphanet Journal of Rare Diseases

Fig. 7

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

Fig. 7

Effect of α-LA supplementation on mitochondrial lipoylated proteins and PDH activity. Control and PKAN fibroblasts (P1, P2, P3) were treated with α-LA at 10 μM for 20 days. a Representative image of lipoylated proteins expression levels assessed by Western blotting. b Densitometry of the Western blotting. c PDH activity in whole cellular extracts was measured as described in Material and Methods. Data represent the mean ± SD of two separate experiments. ****p < 0.0001 between PKAN patients and controls; ##p < 0.005, ###p < 0.001 ####p < 0.0001 between untreated and treated cells

Back to article page

Orphanet Journal of Rare Diseases

ISSN: 1750-1172