FIG. 3.

Import of Pex5 into peroxisomal ghosts in pex mutant cells. (A) Pex5 import assay was performed using several pex mutants defective in matrix protein import. ³⁵S-Pex5 was incubated with PNS from the CHO pex1 mutant ZP107, pex6 ZP164, or pex26 ZP167 in the presence of 1 mM ATP plus ARS. Imported ³⁵S-Pex5 was verified as described in the legend to Fig. 1A. (B) The ³⁵S-Pex5 import assay was likewise carried out using PNS of human fibroblasts from a normal control and patients with PBD of PEX1-defective CG1 (CG-E), PEX6-deficient CG4 (CG-C), or PEX26-impaired CG8 (CG-A). (C) The ³⁵S-Pex5 import assay was performed as for panel A, using CHO-K1-derived PNS in the presence of purified recombinant Pex5 (rPex5) as indicated. Only the proteinase K-treated organelle fractions are shown.