Regeneration-blocked mdx muscle: in vivo model for testing treatments
✍ Scribed by John G. Quinlan; Denise Cambier; Sean Lyden; Arif Dalvi; Ram K. Upputuri; Peter Gartside; Scott E. Michaels; David Denman
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 222 KB
- Volume
- 20
- Category
- Article
- ISSN
- 0148-639X
No coin nor oath required. For personal study only.
✦ Synopsis
We have refined the mdx mouse as a clinical model for Duchenne dystrophy. Our power estimates, primary measures, regular sacrifice intervals, and quality checks constitute a high-speed, low-cost system for preclinically testing therapies designed to slow muscle destruction in Duchenne dystrophy. Irradiated (18 Gy) and contralateral shielded anterior tibial muscles were studied in 21-day-old mdx and normal mice at the time of irradiation and 4, 8, 12, 16, and 20 weeks thereafter. Regeneration-blocked mdx (irradiated) muscle expressed muscular dystrophy as progressive wasting after a brief (4 week) period of growth. Regenerationblocked normal muscle showed stunted growth but neither progressive wasting nor microscopic pathological changes.