Figure 1. NT-1654 induces ectopic AChR clusters.

A: Dose response curve of AChR-aggregating activity of NT-1654 in vitro. NT 1654 was applied on C2C12 myotubes and induced AChR clusters at the concentrations indicated in Fig. S2A. The area of AChR clusters per myotube was measured and normalized to the area of the myotubes. Percentage clustering activity was calculated by the mean value of 108, 36 and 12 nM to 100%. Data points represent mean ± standard error of triplicate cultures with 10 myotube segments counted in each. The curve was fitted to the data points with a sigmoidal regression. The EC₅₀ was determined from the fit as 440 pM and indicated by the dotted lines. B: NT-1654 induces ectopic AChR clustering in vivo. NT-1654 induced ectopic AChR clusters in 6-days denervated soleus and EDL muscles. The ectopic AChR clusters were mainly present in the distal ends of soleus muscle whereas they were mainly at the NMJ zone of EDL muscle. No or only few clusters were observed upon injection of PBS. There is no obvious difference between 10 mg/kg and 1 mg/kg groups. Scale bar: 50 µm.