Vulnerability to NMJ denervation is more pronounced in distal appendicular muscles and can be prevented by AAV9-IGHMBP2 treatment. Quantification of NMJ pathology showing percentages of fully innervated, partially innervated, and fully denervated end plates from 8-week-old wild type, nmd, and nmd + AAV9-IGHMBP2 treated mice. (A) Quantification of NMJ pathology in resistant (cLAL and masseter) and slightly vulnerable (LC and SC) muscles from the neck. (B) Quantification of NMJ pathology in resistant (diaphragm and SPI) and vulnerable (LD and TVA) muscles from the trunk. (C) Quantification of NMJ pathology in vulnerable (gastrocnemius, TA, FDB-2, FDB-3, and FDB-4) and resistant (EDL and lumbricals) muscles from distal appendages. Quantification of NMJ pathology also shows that AAV9-IGHMBP2 treatment of nmd mice significantly reduces NMJ pathology in all muscle groups analyzed (A, B, and C). Data were analyzed by one-way ANOVA followed by a Bonferroni post hoc test for multiple comparisons. Data expressed as mean ± SEM. *P < 0.05, **P < 0.001, and ***P < 0.0001. n.s., not significant. n = 3 animals per treatment.