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Fig. 7 | Stem Cell Research & Therapy

Fig. 7

From: Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities

Fig. 7

Peroxisome assembly in OPCs expressing the GFP-PTS1 reporter gene. Cells were immunostained with antibodies against PGFR-alpha (red) and nuclei were counterstained with DAPI (blue). As indicated by GFP-positive puncta of appropriate size relative to nuclei, GFP-PTS1 was imported into peroxisomes in control cells (top row) whereas in the marked PBD-ZSD patient cells (bottom two rows), GFP-PTS1 showed cytoplasmic localization, reflecting a peroxisome assembly defect. Arrows highlight cells co-expressing PGFR-alpha and GFP-PTS1

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Stem Cell Research & Therapy

ISSN: 1757-6512