Table 3.
Respiratory Chain Enzyme Activity in Muscle Biopsy Samples from Finnish Patients with GRACILE Syndrome
|
Activity (Ratio to CS)b(nmol/min/mg mitochondrial protein) |
|||||
| Samplea | Complex I + III | Complex II + III | Complex II | Complex IV | CS |
| Patient B | 65 (.13) | 89 (.18) | 188 (.37) | 1,169 (2.31) | 506 |
| Patient C | 76 (.11) | 154 (.23) | 195 (.29) | 3,093 (4.54) | 682 |
| Patient D | 84 | 81 | 249 | 1,815 | NDc |
| Patient E | 132 (.21) | 163 (.26) | 101 (.16) | 1,929 (3.08) | 626 |
| Patient F | 218 (.27) | 61 (.07) | 154 (.19) | 2,219 (2.72) | 816 |
| Control (n=7)d | 140 ± 70 (.14) | 197 ± 88 (.20) | 162 ± 51 (.17) | 2,521 ± 759 (2.60) | 969 ± 243 |
a
Samples are isolated mitochondria from muscle biopsy specimens.
b
Activity measurement performed as described by Majander et al. (1995).
c
ND = not determined.
d
Data are mean ± SD (ratio to CS calculated from controls' mean).