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Beyond mice: Emerging and transdisciplinary models for the study of early-onset myopathies

Abstract : The use of the adapted models to decipher patho-physiological mechanisms of human diseases is always a great challenge. This is of particular importance for early-onset myopathies, in which pathological mutations often impact not only on muscle structure and function but also on developmental processes. Mice are currently the main animal model used to study neuromuscular disorders including the early-onset myopathies. However strategies based on simple animal models and on transdisciplinary approaches exploring mechanical muscle cell properties emerge as attractive, non-exclusive alternatives. These new ways provide valuable opportunities to improve our knowledge on how mechanical, biochemical, and genetic/epigenetic cues modulate the formation, organization and function of muscle tissues. Here we provide an overview of how single cell and micro-tissue engineering in parallel to non-mammalian, Drosophila and zebrafish models could contribute to filling gaps in our understanding of pathogenic mechanisms underlying early-onset myopathies. We also discuss their potential impact on designing new diagnostic and therapeutic strategies.
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https://hal.archives-ouvertes.fr/hal-01545055
Contributor : Thomas Boudou <>
Submitted on : Thursday, June 22, 2017 - 1:54:03 PM
Last modification on : Wednesday, July 28, 2021 - 9:57:58 AM

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Krzysztof Jagla, Benoit Kalman, Thomas Boudou, Sylvie Hénon, Sabrina Batonnet-Pichon. Beyond mice: Emerging and transdisciplinary models for the study of early-onset myopathies. Seminars in Cell and Developmental Biology, Elsevier, 2017, 64, pp.171 - 180. ⟨10.1016/j.semcdb.2016.09.012⟩. ⟨hal-01545055⟩

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