| First Author | Granchelli JA | Year | 1995 |
| Journal | J Neurol Sci | Volume | 131 |
| Issue | 1 | Pages | 1-7 |
| PubMed ID | 7561939 | Mgi Jnum | J:26738 |
| Mgi Id | MGI:74171 | Doi | 10.1016/0022-510x(95)00089-k |
| Citation | Granchelli JA, et al. (1995) Duchenne-like myopathy in double-mutant mdx mice expressing exaggerated mast cell activity. J Neurol Sci 131(1):1-7 |
| abstractText | Dystrophin-deficient female mdx mice were bred with male Tsk+/+ pa mice to examine the role played by mast cells in the pathophysiology of dystrophin deficiency. Resultant mdx/Tsk double-mutant mice were then examined functionally, biochemically, and histologically. While mdx mice remained as strong as their normal counterparts, mdx/Tsk double-mutant mice became progressively weak with age. Serum creatine kinase activity was significantly elevated in both mdx and mdx/Tsk double-mutant mice over normal controls. However, mast cell-derived plasma tryptase activity was consistently higher in the double-mutant than in mdx mice. In addition, histological examination of gastrocnemius muscle revealed that while necrosis was persistent in both strains of mdx mice from 2 to 8 weeks of age, regeneration was significantly reduced in the double-mutant mice. Of particular interest was the fact that necrosis in the mdx/Tsk double mutant exceeded mdx values at 8 weeks of age, corresponding approximately with a second peak in tryptase activity. Therefore, heightened mast cell activity appears to elicit in the dystrophin-deficient mdx mouse a myopathy not unlike the human Duchenne disease. |
