ZFIN ID: ZDB-PUB-121121-7
Zebrafish models flex their muscles to shed light on muscular dystrophies
Berger, J., and Currie, P.D.
Muscular dystrophies are a group of genetic disorders that specifically affect skeletal muscle and are characterized by progressive
muscle degeneration and weakening. To develop therapies and treatments for these diseases, a better understanding of the molecular
basis of muscular dystrophies is required. Thus, identification of causative genes mutated in specific disorders and the study
of relevant animal models are imperative. Zebrafish genetic models of human muscle disorders often closely resemble disease
pathogenesis, and the optical clarity of zebrafish embryos and larvae enables visualization of dynamic molecular processes
in vivo. As an adjunct tool, morpholino studies provide insight into the molecular function of genes and allow rapid assessment
of candidate genes for human muscular dystrophies. This unique set of attributes makes the zebrafish model system particularly
valuable for the study of muscle diseases. This review discusses how recent research using zebrafish has shed light on the
pathological basis of muscular dystrophies, with particular focus on the muscle cell membrane and the linkage between the
myofibre cytoskeleton and the extracellular matrix.
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