PUBLICATION
The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
- Authors
- Winder, S.J., Lipscomb, L., Parkin, A.C., and Juusola, M.
- ID
- ZDB-PUB-111205-3
- Date
- 2011
- Source
- PLoS Currents 3: RRN1286 (Journal)
- Registered Authors
- Keywords
- none
- MeSH Terms
- none
- PubMed
- 22130468 Full text @ PLoS Curr.
Citation
Winder, S.J., Lipscomb, L., Parkin, A.C., and Juusola, M. (2011) The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish. PLoS Currents. 3:RRN1286.
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.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping