Table 2.
Respiratory chain complex activities in Patients I and II
| Reference interval |
||||
|---|---|---|---|---|
| Patient I | Patient II | Mean | Range | |
| Polarography | nmol/min per mg protein | |||
| Substrate | ||||
| Pyruvate + malate | 51 | 52 | 98 | 86–116 |
| Glutamate + malate | 50 | 59 | 107 | 86–125 |
| Palmitoylcarnitine + malate | NA | 42 | 63 | 30–84 |
| Succinate + rotenone | 90 | 94 | 99 | 76–117 |
| Ascorbate + TMPD | 155 | 160 | 251 | 207–302 |
| Enzyme activities | μmol/min per mg protein | |||
| NADH/ferricyanide reductase | 4.0 | 3.5 | 6.7 | 4.5–8.6 |
| Succinate/cytochrome c reductase | 0.11 | 0.19 | 0.30 | 0.16–0.40 |
| Citrate synthase | 2.2 | 1.5 | 2.4 | 2.0–3.5 |
| L/min per mg protein | ||||
| Cytochrome c oxidase | 5.0 | 5.6 | 11 | 6.1–15 |
NA, not analyzed; TPMD, N,N,N′,N′-Tetramethyl-p-phenylenediamine.