Table 1.
Characteristics of Induced Dystrophin Isoforms
| Dystrophin Isoforms | Skipped Exons | Dystrophin Protein Level | Dystrophin Immunofluorescent Intensity | β-Dystroglycan Immunofluorescent Intensity | Centrally Nucleated Fibers (Total Fibers Counted) | Fibrosis | Muscle Pathology |
|---|---|---|---|---|---|---|---|
| C57 | |||||||
| Sham-treated | N.A | 100% | 100% | 100% | 0 (538) | 36.10% | – |
| Del23 | 23 | 96.7% | 76.7% | 88.4% | 1.79% (502) | 54.98% | + |
| Del56/57 | 56+57 | 82.3% | 77.4% | 81.0% | 1.84% (489) | 51.33% | + |
| Del58/59 | 58+59 | 27.5% | 48.5% | 71.3% | 25.75% (532) | 80.99% | + + + + |
| Del70 | 70 | 31.8% | 44.1% | 57.4% | 26.85% (517) | 85.41% | + + + + |
| mdx | |||||||
| Sham-treated | N.A | 0 | 30.2% | 56.1% | 28.14% (501) | 100% | + + + + + |
| Del23 | 23 | 81.4% | 79.2% | 78.0% | 6.12% (555) | 63.59% | + + |
| Del23/56/57 | 23+56+57 | 67.5% | 77.3% | 74.5% | 4.60% (717) | 59.9% | + + |
| Del23/58/59 | 23+58+59 | 22.3% | 44.4% | 59.9% | 19.20% (552) | 88.97% | + + + + |
| Del23/70 | 23+70 | 10.1% | 35.8% | 58.2% | 23% (500) | 81.21% | + + + + |
Dystrophin protein level was measured by densitometry and compared to sham-treated C57 (set as 100%) and sham-treated mdx mice (set as 0). Mean immunofluorescent intensity was measured by ImageJ to show the expression level of the dystrophin and β-dystroglycan; immunofluorescence intensity was normalized to sham-treated C57. Total muscle fibers and central nucleated fibers were counted and the percentage of fibers with central nucleation was shown. The degree of fibrosis was determined by ImageJ measuring the intensity of collagen-rich fibrotic regions in blue and was normalized to sham-treated mdx; “+” indicates the severity of muscle pathology; “+ + + + +” indicates the most severe pathology; “–” indicates negative; Del: deletion; N.A., not applicable.