Key result
A miniaturized 3D myotube culture chip successfully modeled LMNA-related Congenital Muscular Dystrophy by developing mutant 3D myotubes displaying contractile dysfunction using minimal biological material.
Why the study?
Existing 3D organ-on-chip models for quantifying skeletal muscle contraction require substantial biological material, limiting their use with scarce patient samples.
The miniaturized 3D myotube chip provides a high-yield, low-input in vitro model for studying muscle contraction and nuclear morphology in muscular dystrophies like L-CMD.
Enables efficient in vitro modeling of L-CMD contractile defects; leaves open human validation and therapeutic translation.
Quantification of skeletal muscle functional contraction is essential to assess the outcomes of therapeutic procedures for muscular disorders. Muscle three-dimensional “Organ-on-chip” models usually require a substantial amount of biological material, which is problematic in the context of limited patient sample. Here we developed a miniaturized 3D myotube culture chip with contraction monitoring capacity. Optimized micropatterned substrate design enabled to obtain high culture yields in tightly controlled microenvironments. Spontaneous contractions in myotubes derived from primary human myoblasts were observed. Analysis of nuclear morphology confirmed a similar organization between obtained myotubes and in vivo myofibers. LMNA -related Congenital Muscular Dystrophy (L-CMD) was modelled with successful development of mutant 3D myotubes displaying contractile dysfunction. This technology can thus be used to study contraction characteristics and evaluate how diseases affect muscle organization and force generation. Importantly, it requires significantly fewer starting materials than current systems, which should allow to substantially improve drug screening capability.
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Rose et al. (2021) studied Muscular dystrophies (LMNA-related Congenital Muscular Dystrophy). Miniaturized 3D myotube culture chip was evaluated on Development of mutant 3D myotubes displaying contractile dysfunction. A miniaturized 3D myotube culture chip successfully modeled LMNA-related Congenital Muscular Dystrophy by developing mutant 3D myotubes displaying contractile dysfunction using minimal biological material.
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