Table 2.
Percentage of ApoCIII isoforms⁎ | ApoCIII-0 (%) | ApoCIII-1 (%) | ApoCIII-2 (%) |
---|---|---|---|
Patient, sample 1 | 5.3 | 68.4 | 26.4 |
Patient, sample 2 | 6.5 | 60.4 | 33.2 |
Patient, sample 3 | 9.7 | 56.5 | 33.7 |
CDG-IIf patient | 6.0 | 78.0 | 16.0 |
Reference range (age: 1–18 yr) | 0–11.6 | 33.1–66.9 | 27.4–60.0 |
Patient's mother | 8.1 | 59.5 | 32.4 |
Reference range (age: >18 yr) | 2.6–18.9 | 42.9–69.2 | 23.2–50.0 |
Three independently collected serum samples of the patient and samples of the patient's mother were subjected to isoelectrical focusing. Results obtained for a CDG-IIf patient26 are shown for comparison. The samples of the patient and her mother show isoform distributions that are within the respective reference ranges, whereas the CDG-IIf results indicate hyposialylation of ApoCIII as seen from the shift of disialylated ApoCIII-2 to monosialylated ApoCIII-1.