Fig. 2

Expression of full-length nebulin and Z-disk fragment in AAV-treated muscles. a Example Western blot using SH3 antibody that labels both full-length and the Z-disk AAV nebulin. Full-length nebulin is only detected in the CTRL mice, in both the vehicle-treated (LTC and LEDL) and AAV-treated muscles (RTC and REDL). The Z-disk fragment is detected in AAV-treated muscles (RTC and REDL) of both genotypes. (Lane marked with ‘C’: AAV-treated muscle that was loaded on all gels and that functioned as a universal control sample allowing for comparison of multiple blots.). b, c Z-disk fragment expression in vehicle-treated and AAV-treated muscles in CTRL (b) and Neb cKO (c) mice. Repeated-measure 2-way ANOVA shows that Z-disk fragment is significantly increased in AAV-treated CTRL and Neb cKO muscles. Significance between vehicle-and AAV-treated muscles marked with #. d Full-length nebulin expression in vehicle-treated and AAV-treated muscles in CTRL mice. Repeated-measure 2-way ANOVA shows that the treatment has a significant effect (*) and that AAV-treated TC muscles experience a significant loss of full-length nebulin (#). e Comparison of Z-disk fragment expression in AAV-treated Neb cKO mice to full-length nebulin in vehicle-treated CTRL mice. Treatment results in a Z-disk fragment expression that is comparable to full-length nebulin in CTRL mice, in both TC and EDL. (Muscles treated with the 1× AAV dose). (n = 6, 8 mice)