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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

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

Fig. 2

Assembly and phosphorylation of vimentin and GFAP. a-d Immunocytochemical analysis of vimentin (green) indicates altered vimentin arrangement in cells bearing a NPC1 mutation. These cells display longer and thicker vimentin bundles (arrows), arranged in a criss-crossed manner (asterik), in comparison to control cells. e-h Similar observations were made in cells stained for GFAP red). Nuclei are stained by DAPI (blue). Scale 10 μm. i, j Amount of phosphorylated vimentin (p-vimentin) and phosphorylated GFAP (p-GFAP) was quantified by semi-quantitative western blot. All NPC1 mutant cell lines displayed a significantly reduced amount of p-vimentin and p-GFAP indicating a hypo-phosphorylation of the IFs. (p-vimentin: N = 6–8, n = 26–40; p-GFAP: N = 5–7, n = 18–31). Examples of according western blot bands are shown below the bar graphs

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