Somatic CAG expansion is suppressed in <i>R6/2</i> mice by XJB-5-131 treatment.

<p>The CAG repeat lengths in the striatum (A) and cerebellum (B) of <i>R6/2</i> mice at 8 or 12 weeks with treatment beginning at 4 weeks. Expansions were observed as a shift in the size distribution of CAG lengths for each mouse (red) compared to the size at weaning (blue). Treatment with XJB-5-131 suppresses expansion in both brain regions. The gender of the mice is designated by ‘M’ (male) or ‘F’ (female). (C) The impact of XJB-5-131 on expansion in the striatum as determined using a quantile analysis [<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0194580#pone.0194580.ref010" target="_blank">10</a>]. The combined size distribution from the striatum in treated and untreated groups was divided into 99 cells. Shown is the size differences in untreated v. treated striatum along the distribution at 12 weeks. Red are cells in which there was a significant increase in the untreated versus the treated (p < 0.05). Blues are cells with no significant increases. Bracketed lines represent 1 SE (Standard Error of the Mean). The significance is listed for each cell in <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0194580#pone.0194580.s005" target="_blank">S2</a> and <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0194580#pone.0194580.s006" target="_blank">S3</a> Tables. (n = 3 mice per treatment group).