Figure 2.

Expression of PEX1 rescues PTS1-mediated import in selected PEX6-deficient cells. Human fibroblasts from a patient with NALD adrenoleukodystrophy (PBD102; A–C) and a patient with ZS (PBD105; D–F) were transfected with pcDNA3 (A and C), derivatives of this plasmid designed to express the PEX1 (B and E), or PEX6 (C and F) cDNAs. Two days after transfection, the cells were processed for indirect immunofluorescence to determine the subcellular distribution of catalase, a PTS1-containing protein. Note that PEX1 expression mediated catalase import in the PBD102 cell line (B) but not PBD105 cells (E) whereas transfection with the expression vector alone had no effect on catalase import in either line. Width of each image panel is 190 μm.