Journal
JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY
Volume 36, Issue 2, Pages 155-167Publisher
SPRINGER
DOI: 10.1007/s10974-015-9406-4
Keywords
Duchenne muscular dystrophy; Mdx mouse; Calcium; Inflammation; Genetic therapy; Corticosteroids
Categories
Funding
- Department of Physiology, UCC
- Muscular Dystrophy Ireland
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Duchenne muscular dystrophy (DMD) is a fatal X-chromosome linked recessive disorder caused by the truncation or deletion of the dystrophin gene. The most widely used animal model of this disease is the dystrophin-deficient mdx mouse which was first discovered 30 years ago. Despite its extensive use in DMD research, no effective treatment has yet been developed for this devastating disease. This review explores what we have learned from this mouse model regarding the pathophysiology of DMD and asks if it has a future in providing a better more thorough understanding of this disease or if it will bring us any closer to improving the outlook for DMD patients.
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