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Advances in the Understanding of Skeletal Myopathies from Zebrafish Models

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Zebrafish, Medaka, and Other Small Fishes

Abstract

Skeletal muscle diseases, or myopathies, are a diverse group of disorders that range in severity from mild muscle weakness to lethality and in onset from in utero to late adulthood. Whilst in some cases the genetic basis of these diseases is known, understanding of the mechanism underlying muscle weakness is often lacking, and there are no effective treatments for these diseases. Zebrafish (Danio rerio) are well established as a model system and offer many advantages in terms of time, cost and ease of experimental manipulation, and in vivo tracking of pathology, for the study of muscle. Both the process of muscle development and muscle function are highly conserved throughout evolution, and, as such, zebrafish muscle has remarkable structural and molecular similarities to that of human and is highly suited to the investigation of skeletal myopathies.

Zebrafish models have been widely applied to the evaluation of potential myopathy disease variants, meeting a growing need for rapid functional analysis given the increasing application of high-throughput sequencing. Many of the models that have been generated, across the range of myopathy subtypes, have been characterised in detail and present pathologies that are strikingly similar to those observed in patients. Research using these models has resulted in significant contributions to our understanding of disease biology and has identified potential therapies. Here we provide a review of zebrafish skeletal myopathy models, detail the advances they have made to the field and highlight areas where they are poised to significantly contribute in the future.

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  1. School of Biological Sciences, Monash University, Melbourne, Australia

    Emily Claire Baxter & Robert J. Bryson-Richardson

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    Hiromi Hirata

  2. Institute for Frontier Life and Medical Sciences, Kyoto University, Kyoto, Japan

    Atsuo Iida

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Baxter, E.C., Bryson-Richardson, R.J. (2018). Advances in the Understanding of Skeletal Myopathies from Zebrafish Models. In: Hirata, H., Iida, A. (eds) Zebrafish, Medaka, and Other Small Fishes. Springer, Singapore. https://doi.org/10.1007/978-981-13-1879-5_9

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