PUBLICATION

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

Authors
Plantié, E., Migocka-PatrzaBek, M., Daczewska, M., Jagla, K.
ID
ZDB-PUB-150411-4
Date
2015
Source
Molecules   20: 6237-6253 (Review)
Registered Authors
Keywords
none
MeSH Terms
  • Animals
  • Disease Models, Animal
  • Drosophila
  • Humans
  • Muscle, Skeletal/metabolism*
  • Muscle, Skeletal/pathology
  • Muscular Dystrophy, Duchenne/genetics*
  • Muscular Dystrophy, Duchenne/metabolism
  • Muscular Dystrophy, Duchenne/pathology
  • Myotonic Dystrophy/genetics*
  • Myotonic Dystrophy/metabolism
  • Myotonic Dystrophy/pathology
  • Zebrafish
PubMed
25859781 Full text @ Molecules
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.
Genes / Markers
Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping