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Molecules 2015, 20(4), 6237-6253; doi:10.3390/molecules20046237

Model Organisms in the Fight against Muscular Dystrophy: Lessons from Drosophila and Zebrafish

1
GReD (Genetics, Reproduction and Development laboratory), INSERM U1103, CNRS UMR6293, University of Clermont-Ferrand, 28 place Henri-Dunant, 63000 Clermont-Ferrand, France
2
Department of Animal Developmental Biology, Institute of Experimental Biology, University of Wroclaw, 21 Sienkiewicza Street, 50-335 Wroclaw, Poland
These authors contributed equally to this work.
*
Author to whom correspondence should be addressed.
Academic Editor: Leonidas A. Phylactou
Received: 24 February 2015 / Revised: 31 March 2015 / Accepted: 1 April 2015 / Published: 9 April 2015
View Full-Text   |   Download PDF [765 KB, uploaded 9 April 2015]

Abstract

Muscular dystrophies (MD) are a heterogeneous group of genetic disorders that cause muscle weakness, abnormal contractions and muscle wasting, often leading to premature death. More than 30 types of MD have been described so far; those most thoroughly studied are Duchenne muscular dystrophy (DMD), myotonic dystrophy type 1 (DM1) and congenital MDs. Structurally, physiologically and biochemically, MDs affect different types of muscles and cause individual symptoms such that genetic and molecular pathways underlying their pathogenesis thus remain poorly understood. To improve our knowledge of how MD-caused muscle defects arise and to find efficacious therapeutic treatments, different animal models have been generated and applied. Among these, simple non-mammalian Drosophila and zebrafish models have proved most useful. This review discusses how zebrafish and Drosophila MD have helped to identify genetic determinants of MDs and design innovative therapeutic strategies with a special focus on DMD, DM1 and congenital MDs. View Full-Text
Keywords: muscular dystrophies; animal models; Drosophila; zebrafish; pathophysiology; therapeutic screens muscular dystrophies; animal models; Drosophila; zebrafish; pathophysiology; therapeutic screens
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Plantié, E.; Migocka-Patrzałek, M.; Daczewska, M.; Jagla, K. Model Organisms in the Fight against Muscular Dystrophy: Lessons from Drosophila and Zebrafish. Molecules 2015, 20, 6237-6253.

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