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. 2017 Dec 20;27(4):679–690. doi: 10.1093/hmg/ddx434

Figure 2.

Figure 2.

Increased vulnerability to NMJ denervation in distal appendicular muscles and prevention with AAV9-IGHMBP2 treatment. Neck (anterior), trunk (middle), and distal appendicular muscles (posterior) were immunostained to label axon (NF-H), axon terminal (SV2), and end plate (AChRs). (Top panel) Images of representative muscle (cLAL) from the neck showing resistance to NMJ denervation in nmd (middle) and nmd+ AAV9-IGHMBP2 treated (right) compared to an unaffected/wild-type control (left) (scale bars = 100 µm). (Middle panel) Images of a representative resistant muscle (diaphragm) from the trunk area in nmd (middle) and nmd+ AAV9-IGHMBP2 treated (right) compared to an unaffected control (left) (scale bars = 30 µm). (Bottom two panels) Representative muscles (gastrocnemius and FDB-2) from distal appendicular area with high vulnerability to NMJ denervation in nmd (middle) compared to control (left) (scale bars = 30 µm). AAV9-IGHMPB2 treatment successfully prevents NMJ denervation in highly vulnerable muscles (bottom right). Maximum projection confocal microscope images taken at 20× magnification.