Table 2.
COX deficiency for dysferlin patients (n = 8) as assessed by sequential COX/SDH histochemistry.
| Investigator | Patient | Total fibres | % COX positive | % Intermediate positive | % Intermediate negative | % COX negative |
|---|---|---|---|---|---|---|
| 1 | DYSF 1 | 1491 | 98.2% | 1.8% | 0.0% | 0.0% |
| DYSF 2 | 577 | 75.4% | 18.4% | 6.1% | 0.1% | |
| DYSF 3 | 963 | 65.5% | 21.9% | 11.9% | 0.6% | |
| DYSF 4 | 1774 | 97.7% | 5.0% | 0.1% | 0.0% | |
| DYSF 5 | 397 | 86.9% | 12.8% | 0.3% | 0.0% | |
| DYSF 6 | 563 | 80.8% | 17.9% | 0.9% | 0.4% | |
| DYSF 7 | 132 | 69.7% | 30.3% | 0.0% | 0.0% | |
| DYSF 8 | 453 | 92.5% | 4.9% | 2.0% | 0.7% | |
| 2 | DYSF 1 | 1357 | 83.6% | 16.3% | 0.07% | 0.0% |
| DYSF 2 | 382 | 71.7% | 26.9% | 1.5% | 0.0% | |
| DYSF 3 | 328 | 65.9% | 30.4% | 3.7% | 0.0% | |
| DYSF 4 | 1131 | 97.1% | 2.6% | 0.2% | 0.1% | |
| DYSF 5 | 395 | 77.7% | 22.0% | 0.3% | 0.0% | |
| DYSF 6 | 726 | 70.4% | 27.1% | 1.9% | 0.6% | |
| DYSF 7 | 135 | 58.5% | 31.1% | 10.4% | 0.0% | |
| DYSF 8 | 463 | 78.2% | 20.3% | 1.2% | 0.4% |
Notes: Fibres were classified as COX positive, COX intermediate positive, COX intermediate negative or negative in order to make it comparable with immunofluorescent assessment.