Figure 6

Combined treatment increases survival motor neuron (SMN) expression and motor neuron numbers in mice with late-onset spinal muscular atrophy (SMA). (A, C) Western blots showing SMN expression in the brain (A) and spinal cord (C) in mice with late-onset SMA that received L-ascorbic acid (L-AA) or combined (n = 4 in each group) treatment. β-Actin was used as an internal control. (B, D) Quantification of SMN protein expression in (A, C). The mean ± SEM was calculated. (E, G) Histological staining of lumbar spinal cord samples on postnatal days (PNDs) 30 (E) and 90 (G) for the wild-type (WT) mice and mice with late-onset SMA that had received different treatments. Scale bar: 100 μm (E and G, upper panel); 50 μm (E and G, lower panel). (F, H) Quantification of motor neuron numbers in the spinal cords obtained from (E) and (G) (n = 3, 40 sections for each group were quantified). The mean ± SEM was calculated. ***P < 0.001, Student's t test.