Table 3.
Complementation of CHO cell mutants and PBD patient fibroblasts by RnPEX3
| CHO mutant | Peroxisomepositive | Patient fibroblasts | Peroxisomepositive | Gene | |
|---|---|---|---|---|---|
| ZPG208 | + | PEX3 | |||
| ZPG209 | + | ||||
| ZP124 | − | (A | VIII) | ||
| B | (VII) | − | PEX10 | ||
| ZP92 | − | (C | IV) | PEX6 | |
| D | (IX) | − | PEX16 | ||
| Z24/ZP107 | − | (E | I) | PEX1 | |
| Z65 | − | (F | X) | PEX2 | |
| ZP139 | − | (II) | PEX5 | ||
| ZP109 | − | (III) | PEX12 | ||
| VI | − | ||||
| G | − | ||||
| ZP128 | − | (H) | PEX13 | ||
| ZP119 | − | J | − | PEX19 | |
| ZPG207 | − | R | XI | − | PEX7 |
| ZP110 | − | PEX14 | |||
| ZP114 | − | ||||
| ZP126 | − | ||||
Peroxisome-deficient CHO mutants (Fujiki, 1997; Tateishi et al., 1997; Kinoshita et al., 1998), including ZPG208 and ZPG209 (Ghaedi et al., 1999b), CG-A ZP124 (Ghaedi et al., 1999a), PEX6-defective ZP92 (Shimozawa et al., 1992; Tsukamoto et al., 1995), PEX1-defective ZP107 (Okumoto et al., 1997; Tamura et al., 1998), PEX2-impaired Z65 (Tsukamoto et al., 1991, 1994b), PEX5-impaired ZP139 (Otera et al., 1998), PEX12-defective ZP109 (Okumoto et al., 1997, 1998b; Okumoto and Fujiki, 1997), PEX13-impaired ZP128 (Toyama et al., 1999), PEX19-impaired ZP119 (Kinoshita et al., 1998; Matsuzono et al., 1999), PEX14-deficient ZP110 (Tateishi et al., 1997; Shimizu et al., 1999), ZP114 (Tateishi et al., 1997), and ZP126 (Ghaedi et al., 1999a), as well as patient fibroblasts of several CGs shown not to be represented by CHO mutants (Ghaedi et al., 1999b), i.e., CGs B (patient PBDB-01) (Okumoto et al., 1998a), D (patient PBDD-01) (Honsho et al., 1998), and G (patient 2) (Poulos et al., 1995; Ghaedi et al., 1999b) of Gifu University (Gifu, Japan) and CG-VI (Shimozawa et al., 1992) of Kennedy-Krieger Institute (Baltimore, MD), were transfected with pcDNA3.1/Zeo·RnPEX3 and examined for peroxisome assembly by immunostaining with antisera to rat and human catalase, respectively, at 4 d after transfection. PEX7-defective ZPG207 (Ghaedi et al., 1999b) and fibroblasts from a patient with rhizomelic chondrodysplasia punctata (Ghaedi et al., 1999b) were likewise transfected and stained with anti-rat thiolase antibody. Cells of CG in parenthesis were not used in this experiment. +, peroxisomes were complemented; −, not complemented.