Figure 1
Preliminary characterization of the SBMA mouse model.
(a) Spinal cord and quadricep cross sections of control and AR113Q male mice at 24 weeks of age (corresponding to symptomatic stage). Nissl staining of lumbar spinal cord shows the cell body of lower motor neurons. Hematoxylin-Eosin staining of quadriceps underscores a marked decrease in fiber size of AR113Q mice compared to control mice. (b) Left Panel: average number of total motor neurons in the lumbar spinal cord of control and AR113Q mice at 24 weeks of age (control mice, n = 5; AR113Q, n = 6); Right panel: average number of motor neurons for each slice (number of slices = 7). (c) Measurement of body weight in control and AR113Q mice at 9 and 24 weeks of age (control mice, n = 5; AR113Q, n = 20). (d) Grip strength test in control and AR113Q mice at 9 and 24 weeks of age (control mice, n = 5; AR113Q, n = 15). **p < 0.001 vs. 24 weeks control mice; °°p < 0.001 vs. 9 weeks AR113Q mice. (e) Rotarod test in control and AR113Q male mice at 24 weeks of age. *p < 0.05 vs. 24 weeks control mice (control mice, n = 5; AR113Q, n = 15). (f) Filter retardation assay in spinal cord and quadriceps of control and AR113Q male mice at 24 weeks. Androgen receptor AR aggregation was visible only in ARQ113 quadriceps at 24 weeks of age. **p < 0.001 vs. 24 weeks quadriceps of control mice.
