Fig. 7
From: Sarcolemma resilience and skeletal muscle health require O-mannosylation of dystroglycan
Restoring expression of Pomt1 in Pomt1skm mice limits skeletal muscle pathology. Four-week-old Pomt1skm mice were administered AAV2/9-MCK-mPOMT1 (AAV-POMT1) via retro-orbital intravenous injection and effects were compared to control or Pomt1skm mice. A Immunofluorescence for matriglycan (recombinant rabbit IIH6 antibody), the c-terminus of β-DG (MANDA G2 7D11 antibody), dystrophin, and perlecan in transverse cross-sections of gastrocnemius muscles. Images shown at 40X magnification. Scale bar = 100 µm. B Body weights of mice sixteen-weeks after AAV injection. ** < 0.007. C Forelimb grip strength four-weeks after AAV-POMT1 injection. *** = 0.0009; **** < 0.0001. D-F. Sixteen-weeks after AAV-POMT1 injection, in vivo plantar flexor torque production was assessed. D. isometric torque production with the ankle positioned at 90-degrees. * = 0.0158; *** = 0.0001. E. Response of plantar flexors to repeated eccentric contractions. * < 0.05; *** < 0.001. N = 8 CON; 5 Pomt1skm; 5 Pomt1skm + AAV. F. Serum creatine kinase (CK) activity under baseline conditions to indirectly assess the extent of sarcolemma damage. *** = 0.0006; **** < 0.0001. P-values for all data determined by one-way ANOVA with Tukey’s multiple comparisons test. Data are expressed as mean ± standard deviation