Table 3.
Specific enzyme analysis of ADOA patient and control mitochondria
|
Mean specific enzyme activities nmol/min/mg mitochondrial protein |
||||
| Complex I | Complex III | Complex IV | CSa | |
| aOPA1+ (n = 6) | ||||
| P2 | 82 | 1076 | 884 | 1410 |
| P3 | 274 | 976 | 568 | 2074 |
| P4 | 294 | 928 | 1005 | 1693 |
| P6 | 86 | 1313 | 970 | 918 |
| P7 | 52 | 1078 | 974 | 846 |
| P8 | 122 | 845 | 828 | 1018 |
| Mean ± SD | 152 ± 105 | 1036 ± 162 | 872 ± 162 | 1327 ± 488 |
| aOPA1- (n = 10) | ||||
| Mean ± SD | 190 ± 144 | 945 ± 174 | 810 ± 204 | 1154 ± 650 |
| Control (n = 10) | ||||
| Mean ± SD | 170 ± 64 | 886 ± 179 | 893 ± 271 | 1304 ± 364 |
aAbbreviations used above are CS, citrate synthase, OPA1+, OPA1-positive patients, and OPA1-, OPA1-negative patients