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Fig. 6 | Orphanet Journal of Rare Diseases

Fig. 6

From: Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis

Fig. 6

Effect of PMA on cholesterol amounts. a-h Filipin staining (blue) was used to assess cholesterol content and demonstrated typical accumulations in all NPC1 mutant cell lines. Cholesterol accumulations were ameliorated after the treatment with PMA, resulting in a Filipin staining pattern comparable to the control cells. Scale 100 μm (i). Accordingly the analysis of the fluorescence intensities revealed a decreased amount of cholesterol (N = 4, n = 40–50). j Similar results are shown for the quantification of the cholesterol amount using the Amplex red assay. The amount decreased significantly to the cholesterol amount of control cells (N = 4–5, n = 6–18). Asterisks above bars indicate significance to untreated control and asterisks above lines indicate significances between treated and untreated cells

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