The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish

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...

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Autores principales: Winder, Steve J, Lipscomb, Leanne, Angela Parkin, Caroline, Juusola, Mikko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Muscular Dystrophy
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3219425/
https://www.ncbi.nlm.nih.gov/pubmed/22130468
http://dx.doi.org/10.1371/currents.RRN1286
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author Winder, Steve J
Lipscomb, Leanne
Angela Parkin, Caroline
Juusola, Mikko
author_facet Winder, Steve J
Lipscomb, Leanne
Angela Parkin, Caroline
Juusola, Mikko
author_sort Winder, Steve J
collection PubMed
description 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.
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spelling pubmed-32194252011-11-29 The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish Winder, Steve J Lipscomb, Leanne Angela Parkin, Caroline Juusola, Mikko PLoS Curr Muscular Dystrophy 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. Public Library of Science 2011-11-17 /pmc/articles/PMC3219425/ /pubmed/22130468 http://dx.doi.org/10.1371/currents.RRN1286 Text en http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Muscular Dystrophy
Winder, Steve J
Lipscomb, Leanne
Angela Parkin, Caroline
Juusola, Mikko
The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title_full The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title_fullStr The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title_full_unstemmed The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title_short The proteasomal inhibitor MG132 prevents muscular dystrophy in zebrafish
title_sort proteasomal inhibitor mg132 prevents muscular dystrophy in zebrafish
topic Muscular Dystrophy
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3219425/
https://www.ncbi.nlm.nih.gov/pubmed/22130468
http://dx.doi.org/10.1371/currents.RRN1286
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