FIGURE 5.

A functional neuromuscular junction (NMJ) model for amyotrophic lateral sclerosis (ALS). Motoneurons (MNs) differentiated from patients with ALS induced pluripotent stem cells (iPSCs) were introduced into a chambered NMJ system and, cocultured with primary myoblasts derived from healthy subjects to encourage NMJ formation. Functional NMJs were detected by recording myofiber contractions (by phase contrast differentials) while electrically stimulating the MNs. NMJs containing ALS MNs demonstrated increased failure of muscle contractions upon repetitive MN stimulations compared to WT controls. This defect was rescued by the application of Deanna Protocol (DP). SKM, skeletal muscle