Table 3.
Biochemical Parameters Correlated with the PEX1 Protein Level
| Biochemical Parameter | Patient 9 | Patient 11/Patient 12 |
| VLCFA C26:C22 ratioa | .12 | .31/.48 |
| DHAPAT activityb (nmol/2h × mg protein) | 4.3 | .1/.1 |
| Catalase import | Partial import into peroxisomes | No import into peroxisomes |
| Age/condition | 6 years, alive, relatively good condition | Died before age 3 mo |
| PEX1 protein level (% of control) | ∼7 | 0/0 |
| Diagnosis | Nonclassical ZS (IRD) | Classical ZS |
a
Control values: mean = .04; range = .03–.07.
b
Control values: 8.5 ± 2.0 nmol/2h × mg protein.