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. 2017 Jul 3;114(29):E5920–E5929. doi: 10.1073/pnas.1701832114

Fig. S4.

Fig. S4.

Neuron loss and storage material accumulation in treated mice at 9 mo. (A) Unbiased optical fractionator counts of neurons at 9 mo in Nissl-stained sections showing that intracranially (IC-AAV2/9), intrathecally (IT-AAV2/9), and combination- (IC/IT-AAV2/9) treated mice have significantly decreased neuron counts in the M1 motor cortex compared with wild-type mice. All treated groups also show significantly decreased counts in the thalamus (VPM/VPL) compared with WT mice. However, there is no change between treatment groups and WT in the ventral horn of the lumbosacral spinal cord (LSC). (B) Thresholding image analysis of autofluorescent storage material is significantly increased in the thalamus in all treated groups compared with WT. In the M1 motor cortex, the IT-AAV2/9 AFSM levels are significantly increased compared with WT. In the spinal cord, AFSM levels in the IC-AAV2/9 spinal cord are significantly increased compared with WT. Dots represent scatterplots of individual animals. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001, one-way ANOVA with post hoc Bonferroni correction. Values shown are mean ± SEM (n = 3 mice per group).