Figure 2.

The Pre- and Post-synaptic Morphological Defects in Type 1 SMA

(A–D) Representative confocal micrographs showing the immunocytochemically labeled NMJ-LSs of iPSC-derived neurons and C2C12 myotubes. (A and B) Representative images of AChR clusters formed by C1 MNs. (C) AChR clusters stained with MHC. (D) NMJ-LSs of patient (P1)- and control (C1)-derived MNs on day 60.

(E) Quantitative immunocytochemical analysis of the α-BTX-positive area (means ± SEM, n = 3, Wilcoxon rank-sum test).

(F) Abnormal NF accumulation (yellow arrows) and poor terminal arborization of MNs in the SMA NMJ-LSs. See also Figure S2.