Table 2.
CHO mutant | Peroxisomepositive clone | Patient fibroblasts from CG | Peroxisomepositive | Gene |
---|---|---|---|---|
ZP119 | 24/30 | J (PBDJ-01) | + | PEX19 |
ZP165 | 21/30 | |||
ZP107 | − | E (I) | − | PEX1 |
ZP139 | − | II | − | PEX5 |
ZP109 | − | III | − | PEX12 |
ZP92 | − | C (IV) | − | PEX6 |
(VI) | ND | |||
B (VII) | − | PEX10 | ||
A (VIII) | − | |||
D (IX) | − | PEX16 | ||
Z65 | − | F (X) | − | PEX2 |
G | − | |||
ZP110 | − | |||
ZP114 | − |
Peroxisome-deficient CHO mutants of 8 CGs (4–8, 13–18) and fibroblasts from 10 CG PBD patients (3, 4, 8, 9) were transfected with pUcD2Hyg⋅HsPEX19 and examined for peroxisome assembly by immunostaining with antisera to rat and human catalase, respectively, at 3 days after transfection. Parentheses indicate fibroblasts of CG not used in this experiment. In CG-J ZP119 and ZP165, peroxisome-positive colonies were counted in 30 colonies; in other cells: +, complemented; −, not complemented; ND, not determined.