FIG 14.
Perturbed neuromuscular structure in HSACrePofut1FF muscle. (A) Confocal image of NMJ staining. Presynaptic motor nerve terminals and axons were stained with synaptophysin and neurofilament (both in green), and postsynaptic membranes were stained with α bungarotoxin (to show AChRs) (red). Merged staining is shown in yellow. HSACrePofut1FF panels show examples of AChR fragmentation (middle panels) and denervation (right panels). The images show 3-month-old diaphragm muscle. Bar, 10 μm. (B and C) Quantification of NMJ ultrastructure changes in 5-month-old HSACrePofut1FF and Pofut1FF diaphragm muscles stained as described for panel A, including measures of the number of AChR-rich fragments per NMJ (B) and the nerve terminal occupancy of postsynaptic AChRs (C). Error bars in panels B and C show SEM (n = 21 to 25 samples per condition). *, P < 0.05; ***, P < 0.001.