Table 3.
Pathological evaluation of Mtm1δ4 tibialis anterior muscles after four doses of treatment with 3E10Fv-MTM1 or vehicle
| Genotype/muscle | Fiber size (μm) | % fibers with internal nuclei | % fibers with subsarcolemmal mitochondrial aggregates | % glycolytic fibers (Type 2B) |
|---|---|---|---|---|
| Light microscopic studies | ||||
| TBS | 24.43 ± 3.00 | 2.98 ± 0.62 | 11.12 ± 2.34 | 86.91 ± 3.25 |
| 3E10Fv-alone | 26.84 ± 1.62 | 4.8 ± 0.28 | 10.11 ± 2.88 | 90.79 ± 2.51 |
| 3E10Fv-MTM1 | 23.62 ± 0.70 | 8.2 ± 2.38 | 10.74 ± 10.40 | 82.17 ± 3.80 |
| Treatment | Fibers quantified | Triads/field | T-Tubules/field | L-Tubules/field |
| Ultrastructural studies | ||||
| TBS | 36 | 4.08 ± 1.02 | 3.83 ± 0.99 | 1.38 ± 0.26 |
| 3E10Fv-alone | 31 | 1.87 ± 0.36 | 1.65 ± 0.34 | 2.55 ± 0.29 |
| 3E10Fv-MTM1 | 33 | 10.12 ± 1.24* | 8.75 ± 1.20* | 2.00 ± 0.29 |
Injected tibialis anterior muscles were evaluated with respect to the characteristic pathological findings associated with myotubularin deficiency, using the same methods employed for Tables 2 and 3. All muscles were collected at 6 weeks of life, after receiving four doses of TBS, 3E10Fv-alone, or 3E10FV-MTM1 over a 2-week period. Data are presented as the mean ± SEM.
*P < 0.05.