Figure 6.
Modeling muscular dystrophies using tissue engineering. (a) 3D artificial skeletal muscle constructs derived from healthy and dystrophic hPSCs. Immunofluorescence for myosin heavy chain (MyHC) on muscle constructs derived from hESCs and dystrophic hiPSCs (DMD, LGMD2D, and skeletal muscle LMNA) differentiated in 3D for 10 days. Nuclei are counterstained with Hoechst. Arrowheads: multinucleated myotubes. Scale bars: top 250 μm, bottom 25 μm. (b) Confocal (z stacks merge) immunofluorescence for DESMIN (myotubes), LAMIN A/C, and EMERIN (nuclear lamina) on hiPSC-derived (healthy and LMNA mutant) artificial muscles. Hoechst: nuclei. Scale bars: 15 μm. Adapted from Maffioletti et al.92.
