Fig. 6.
D-2PMPA treatment selectively improves muscle function and innervation in SOD1G93A mice. A, B Grip strength data from male (A) and female (B) mice. D-2PMPA–treated mice have significantly higher grip strength over 8 weeks or 10 weeks, respectively. Vehicle male n = 20, female n = 18; D-2PMPA male n = 21, female n = 19. Points represent mean ± SEM. Blue points represent SOD1G93A vehicle–treated mouse values. Red points represent SOD1G93A D-2PMPA–treated mouse values. C, H Representative images of WT (male C; female D), vehicle (male E; female F), and D-2PMPA (male G; female H)–treated SOD1G93A gastrocnemius muscle after 4 weeks (male) or 6 weeks (female). Axons stained green. NMJs stained red. Scale bar, 50 µm. Arrows indicate innervated NMJ. Arrowheads indicate denervated NMJs. I, J Quantification of the percentage of innervated NMJs from male (I) and female (J) mice. Bars represent mean ± SEM. Black bars represent WT values. Blue bars represent SOD1G93A vehicle–treated mouse values. Red bars represent SOD1G93A D-2PMPA–treated mouse values. Numbers in parenthesis indicate the total number of NMJs. N = 6/group. *p < 0.05. **p < 0.01. ****p < 0.0001