Fig. 6.
Survival motor neuron (SMN) localization and nuclear complex formation in motor neurons of mice at postnatal day 100. (A) SMN immunohistochemical analysis of lumbar spinal cords from mice derived from SOD1G93A B6SJL mice. In wild-type (WT) mice, SMN shows cytoplasmic and nuclear distribution in NeuN-positive motor neurons, the latter forming gems (arrowhead). In PrP-SMN mice, there are multiple cytoplasmic aggregates of SMN. In SOD1G93A mice, SMN levels are severely reduced and nuclear gems disrupted. In SOD1G93A; PrP-SMN mice, SMN is abnormally localized around the nucleus and gems are disrupted. Scale bar = 50 μm, inset scale bar = 10 μm. (B) Quantification of SMN nuclear gems in motor neurons of mice. Data represent means ± SEM, n = 3 mice per genotype, ** p < 0.01 and *** p < 0.001 using 1-way analysis of variance (ANOVA) with Tukey's posttest.