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.
journal_name
Semin Cell Dev Bioljournal_title
Seminars in cell & developmental biologyauthors
Jagla K,Kalman B,Boudou T,Hénon S,Batonnet-Pichon Sdoi
10.1016/j.semcdb.2016.09.012subject
Has Abstractpub_date
2017-04-01 00:00:00pages
171-180eissn
1084-9521issn
1096-3634pii
S1084-9521(16)30303-2journal_volume
64pub_type
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