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Electron transport chain (ETC) results.
| Patient 1 Liver |
Patient 1 SFC* |
Patient 2 SFC* |
|
|---|---|---|---|
| NADH ferricyanide reductase (complex I) | 19 | 93 | 80 |
| NADH-cytochrome c reductase (complex I + III) | 62 | 160 | 75 |
| Succinate cytochrome c reductase (complex II + III) | 46 | 137 | 99 |
| Succinate dehydrogenase (complex II) | 62 | 114 | 129 |
| Cytochrome c oxidase (complex IV) | 26 | 74 | 62 |
| Citrate synthase | 60 | 86 | 96 |
ETC activities are expressed in % of normal mean.;
SFC : skin fibroblast cells
