Table 3.
Complementation groups (CGs) and PEX genes of peroxisome deficiencies
| Gene | CG | PBD | CHO mutants | Ps-memb. biogenesisa | Peroxin | ||
|---|---|---|---|---|---|---|---|
| US/EU | Japan | (kDa) | Characteristics | ||||
| PEX1 | 1 | E | ZS, NALD*, IRD* | Z24, ZP107 | + | 143 | AAA family |
| PEX2 | 10 | F | ZS, IRD* | Z65 | + | 35 | PMP, RING |
| PEX3 | 12 | G | ZS | ZPG208 | − | 42 | PMP, PMP-DP |
| PEX5 | 2 | ZS, NALD | ZP105*, ZP139 | + | 68 | PTS1 receptor, TPR family | |
| PEX6 | 4(6) | C | ZS, NALD* | ZP92 | + | 104 | AAA family |
| PEX7 | 11 | R | RCDP | ZPG207 | + | 36 | PTS2 receptor, WD motif |
| PEX10 | 7(5) | B | ZS, NALD | + | 37 | PMP, RING | |
| PEX11β | 16 | ZS | + | 28 | PMP | ||
| PEX12 | 3 | ZS, NALD, IRD | ZP109 | + | 40 | PMP, RING | |
| PEX13 | 13 | H | ZS, NALD* | ZP128 | + | 44 | PMP, PTS1-DP, SH3 |
| PEX14 | 15 | K | ZS | ZP110 | + | 41 | PMP, PTS1-DP, PTS2-DP |
| PEX16 | 9 | D | ZS | − | 39 | PMP, PMP-DP | |
| PEX19 | 14 | J | ZS | ZP119 | − | 33 | CAAX motif, PMP receptor |
| PEX26 | 8 | A | ZS, NALD*, IRD* | ZP124, ZP167 | + | 34 | PMP, Pex1p-Pex6p recruiter |
| ZP114 | + | ||||||
*, temperature-sensitive phenotype. aPeroxisomal membrane assembly is normal (+) or impaired (−).
PBD, peroxisomal biogenesis disorders; ZS, Zellweger syndrome; IRD, infantile Refsum disease; NALD, neonatal adrenoleukodystrophy; RCDP, rhizomelic chondrodysplasia punctata; DP, docking protein; PMP, peroxisome membrane protein; TPR, tetratricopeptide repeat.