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. 2015 Aug 26;35(34):12063–12079. doi: 10.1523/JNEUROSCI.0608-15.2015

Figure 2.

Figure 2.

Genetic reduction of IGF-1R expression extends survival and motor behavior and promotes neuroprotection in SMA mice. A, Phenotype of Igf-1r+/− control mice compared with SMA mice and Igf-1r+/− SMA mice at 12 d of age. Scale bar, 1 cm. B, Lifespan of Igf-1r+/+ compared with Igf-1r+/− control and SMA mice (n = 40). C, Weight curve of Igf-1r+/+ and Igf-1r+/− control (left) and SMA (right) mice until SMA death (n = 40). D, Grip time of Igf-1r+/+ and Igf-1r+/− control (left) and SMA (right) mice until SMA death (D; n = 20). E, Total number of squares crossings during 5 min in the open-field test for Igf-1r+/+ and Igf-1r+/− control (left) and SMA (right) mice until SMA death (E; n = 20). FI, Immunodetection of ChAT-positive motor neurons in the lumbar spinal cord (L1–L5) of Igf-1r+/+ (F) and Igf-1r+/− control mice (G) compared with Igf-1r+/+ (H) and Igf-1r+/− SMA mice (I) at 12 d of age (n = 10. Scale bar, 50 μm. J, K, Quantitative analysis of the number (J) and the cell body area (K) of motor neurons per ventral horn in the ventral lumbar spinal cord of Igf-1r+/+ and Igf-1r+/− control mice compared with Igf-1r+/+ and Igf-1r+/− SMA mice at 12 d of age (n = 10). L, M, Western blot analysis (L) and quantification (M) of cleaved caspase-3 protein expression in the ventral lumbar spinal cord of Igf-1r+/+ and Igf-1r+/− control mice compared with Igf-1r+/+ and Igf-1r+/− SMA mice at 12 d of age (n = 4). Dotted lines on Western blot images symbolize some removed interspacing lanes for a side-by-side display of samples from all groups. Data are represented as mean ± SEM and significance is reported versus Igf-1r+/+ control mice (*p < 0.05, **p < 0.01, ***p < 0.001).