Enzymatic activities of mitochondrial respiratory chain complexes
| Assay | Patient I-1 | Patient I-2 | Controls±SD (n=50) |
|---|---|---|---|
| Complex I | 0.047 (24%) | 0.050 (25%) | 0.199±0.043 |
| Complex I+III | 0.087 (40%) | n.d. | 0.217±0.599 |
| Complex II | 0.099 (64%) | 0.091 (59%) | 0.154±0.024 |
| Complex II+III | 0.100 (65%) | 0.053 (34%) | 0.153±0.039 |
| Complex III | 0.934 (46%) | n.d. | 2.01±0.40 |
| Complex IV | 0.270 (21%) | 0.230 (22%) | 1.03±0.24 |
| Complex V | 0.151 (44%) | n.d. | 0.34±0.096 |
*mU/U citrate synthase (% of control).
n.d. not determined.