Figure 7
Combined treatment improves muscle pathology in mice with late-onset spinal muscular atrophy (SMA). (A, B) The tibialis anterior (TA) muscle weight-to-body weight ratio in mice with late-onset SMA in response to different treatments on postnatal days (PNDs) 30 and 90 (n = 3 in each group). The mean ± SEM was calculated. **P < 0.01 and ***P < 0.001, Student's t test. NS = not significant. (C) Histological assessment of hematoxylin and eosin (H&E)-stained TA muscle from PND 30 (upper panel) and 90 (lower panel) wild-type (WT) mice or mice with late-onset SMA that received different treatments. Scale bar: 50 μm. (D) Quantification of the muscle area (μm2) in the PND 30 (left) and 90 (right) mice (n = 3, >500 myofibers for each group were quantified) obtained in (C). The mean ± SEM was calculated. **P < 0.01 and ***P < 0.001, Student's t test. NS = not significant. (E) Staining with the axonal marker neurofilament H (green) and neuromuscular junction (NMJ) marker α-bungarotoxin (α-BTX) (red) revealed NMJs in the treated PND 30 and 90 WT or SMA mice (n = 3). Bar: 50 μm. (F) Quantification of the NMJ area (μm2) in the PND 30 (left) and 90 (right) mice in each group obtained in (E). The mean ± SEM was calculated. **P < 0.01 and ***P < 0.001, Student's t test. NS = not significant.