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PLoS Curr. 2011 Nov 17;3:RRN1286. doi: 10.1371/currents.RRN1286.

The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish.

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  • 1Department of Biomedical Science, University of Sheffield and Centre for Developmental and Biomedical Genetics, Department of Biomedical Science, University of Sheffield.

Abstract

Using sapje zebrafish which lack dystrophin, we have assessed both the quantitation of muscle damage in dystrophic fish, and the efficacy of the proteasomal inhibitor MG132 in reducing the dystrophic symptoms. Fourier analysis of birefringence patterns in normal and dystrophic fish was found to be a simple and reliable quantitative measure of muscle damage. MG132, as in mdx mouse, was found to be effective in reducing muscle damage with an EC50 of 0.4µM. This study adds further to the utility of zebrafish as a model of choice for testing muscular dystrophy therapeutics.

PMID:
22130468
[PubMed]
PMCID:
PMC3219425
Free PMC Article
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