Fig. 8

Peroxisome assembly in hepatocyte-like cells expressing the GFP-PTS1 reporter gene. Cells were immunostained with antibodies against ALB (red) and nuclei were counterstained with DAPI (blue). GFP-positive puncta of appropriate size relative to nuclei in control cells (top strip) indicate peroxisomal import of GFP-PTS1. In the marked patient cells (bottom two rows), GFP-PTS1 had cytoplasmic localization, indicating a peroxisome assembly defect. Arrows highlight cells co-expressing ALB and GFP-PTS1