What has the mdx mouse model of duchenne muscular dystrophy contributed to our understanding of this disease?

Research output: Contribution to journalReview articlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)155-167
Number of pages13
JournalJournal of Muscle Research and Cell Motility
Volume36
Issue number2
DOIs
Publication statusPublished - 1 Apr 2015

Keywords

  • Calcium
  • Corticosteroids
  • Duchenne muscular dystrophy
  • Genetic therapy
  • Inflammation
  • Mdx mouse

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