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Semin Cell Dev Biol. 2017 Apr;64:171-180. doi: 10.1016/j.semcdb.2016.09.012. Epub 2016 Sep 23.

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

Author information

1
Ladybird Team GReD, Univ. Clermont-Ferrand, INSERM U1103, CNRS UMR6293, 63000 Clermont Ferrand, France.
2
Univ. Grenoble Alpes, Grenoble Institute of Technology, CNRS UMR5628 (LMGP), F-38000 Grenoble, France.
3
Matière et Systèmes Complexes, Univ Paris Diderot, Sorbonne Paris Cité, CNRS, UMR 7057, F-75013 Paris, France.
4
Unité de Biologie Fonctionnelle et Adaptative, Univ Paris Diderot, Sorbonne Paris Cité, CNRS, UMR 8251, F-75013 Paris, France. Electronic address: sabrina.pichon@univ-paris-diderot.fr.

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.

KEYWORDS:

Drosophila; Early onset myopathies; Mechanotransduction; Skeletal muscle models; Tissue engineering; Zebrafish

PMID:
27670720
DOI:
10.1016/j.semcdb.2016.09.012
[Indexed for MEDLINE]

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