Table 2.
Complementation of CHO cell mutants and patient fibroblasts by HsPEX1
| CHO mutant | Peroxisome-positive clone | Patient fibroblasts from CG | Peroxisome-positive | Gene |
|---|---|---|---|---|
| ZP107 | 24/30 | E (PBDE-04) (I) | + | PEX1 |
| Z24 | 9/30 | E (PBDE-13) (I) | + | |
| E (PBDE-14) (I) | 6/30 | |||
| ZP139 | − | II | − | PEX5 |
| ZP109 | − | III | − | PEX12 |
| ZP92 | − | C (IV) | − | PEX6 |
| VI | − | |||
| B (VII) | − | |||
| A (VIII) | − | |||
| D (IX) | − | |||
| Z65 | − | F (X) | − | PEX2 |
| G | − | |||
| ZP110 | − | |||
| ZP114 | − |
Peroxisome-deficient CHO mutants (5, 13), including CG-I CHO cell mutants ZP107 (11) and Z24 (9), PEX5-impaired ZP139 (12), PEX12-defective ZP109 (11, 16), PEX6-defective ZP92 (3, 15), and PEX2-impaired Z65 (14, 17, 43), and patient fibroblasts of ten groups of peroxisomal diseases (5)—i.e., CGs A, B, C, D, E (three patients, PBDE-04, PBDE-13, and PBDE-14), F, and G of Gifu University, Japan, and CG-II, -III, and -VI of the Kennedy–Krieger Institute, Baltimore, MD, were transfected with pCMVSPORT⋅HsPEX1 and examined for peroxisome assembly by immunostaining with antisera to rat and human catalase, respectively, at 3 days after transfection. Parentheses indicate cells of CG not used in this experiment. In CG-I cells, ZP107, Z24, and PBDE-14 fibroblasts, peroxisome-positive colonies were counted in 30 colonies; in other cells: +, complemented; −, not complemented.