A human in vitro model of Duchenne muscular dystrophy muscle formation and contractility

• DOI: 10.1083/jcb.201603111
• Publication Date: 2016-10-03T14:15:25Z
• Authors: Alexander P. Nesmith; Matthew A. Wagner; Francesco S. Pasq...; Blakely B. O’Connor; Mark J. Pincus; Paul R. August; Kevin Kit Parker
• Keywords: Contractility; Muscle weakness; ITGA7

Tongue weakness, like all weakness in Duchenne muscular dystrophy (DMD), occurs as a result of contraction-induced muscle damage and deficient repair. Although membrane fragility is known to potentiate injury DMD, whether stem cells are implicated repair remains unclear. We hypothesized that DMD myoblasts less sensitive cues the extracellular matrix designed structure-function relationships healthy muscle. To test this hypothesis, we drew inspiration from tongue engineered contractile human tissues on thin films. On platform, formed fewer smaller myotubes exhibited impaired polarization cell nucleus cytoskeleton when compared with cells. These structural aberrations were reflected their functional behavior, tongues failed achieve same strength structures. data suggest dystrophic may fail organize respect necessary adaptive growth remodeling.