Journal
MUSCLE & NERVE
Volume 44, Issue 4, Pages 553-562Publisher
WILEY
DOI: 10.1002/mus.22125
Keywords
calpain; heart; muscular dystrophy; proteasome; skeletal muscle
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Funding
- NINDS [1U24NS059696-01A1]
- Muscular Dystrophy Association
- National Institutes of Health [1R01HL104129-01]
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Introduction: Previous studies have tested the hypothesis that calpain and/or proteasome inhibition is beneficial in Duchenne muscular dystrophy, based largely on evidence that calpain and proteasome activities are enhanced in the mdx mouse. Methods: mRNA expression of ubiquitin-proteasome and calpain system components were determined using real-time polymerase chain reaction in skeletal muscle and heart in the golden retriever muscular dystrophy model. Similarly, calpain 1 and 2 and proteasome activities were determined using fluorometric activity assays. Results: We found that less than half of the muscles tested had increases in proteasome activity, and only half had increased calpain activity. In addition, transcriptional regulation of the ubiquitin-proteasome system was most pronounced in the heart, where numerous components were significantly decreased. Conclusion: This study illustrates the diversity of expression and activities of the ubiquitin-proteasome and calpain systems, which may lead to unexpected consequences in response to pharmacological inhibition. Muscle Nerve 44: 553-562, 2011
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