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.
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Abbreviations
- Ca2+ :
-
Calcium
- CRF:
-
Corticotrophin releasing factor
- CRFR:
-
CRF receptor
- CRP:
-
C-reactive protein
- GI:
-
Gastrointestinal
- HPA:
-
Hypothalamic-pituitary-adrenal
- IL:
-
Interleukin
- IL-6R:
-
Interleukin-6 receptor
- TTX:
-
Tetrodotoxin
- xIL-6R:
-
Anti-IL-6R
- GRMD:
-
Golden retriever muscular dystrophy
- DAPC:
-
Dystrophin associated protein complex
- ECG:
-
Electrocardiogram
- ACE:
-
Angiotensin-converting enzyme
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The authors have no financial, professional or personal conflicts relating to this publication.
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J.M. was part funded by the Department of Physiology, UCC and Muscular Dystrophy Ireland.
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Manning, J., O’Malley, D. What has the mdx mouse model of duchenne muscular dystrophy contributed to our understanding of this disease?. J Muscle Res Cell Motil 36, 155–167 (2015). https://doi.org/10.1007/s10974-015-9406-4
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DOI: https://doi.org/10.1007/s10974-015-9406-4