Zebrafish models flex their muscles to shed light on muscular dystrophies

J Berger, PD Currie - Disease models & mechanisms, 2012 - journals.biologists.com
Disease models & mechanisms, 2012journals.biologists.com
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 …
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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