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. 2024 Feb 22;147(5):1784–1798. doi: 10.1093/brain/awae063

Figure 2.

Figure 2

CAG repeat instability in zQ175 mice is dependent on Msh3 expression. Representative GeneMapper traces of CAG repeat profiles for brain regions and peripheral tissues from mice at 6 months of age (n = 8/genotype). The modal CAG repeat length in ear samples at 12 days of age (P12) for zQ175 mice was 186 ± 2.3, for the zQ175:Msh3+/− mice was 185 ± 2.4 and for zQ175:Msh3−/− mice was 182 ± 2.0. The representative traces are from the same mouse for each genotype with the CAG repeat mode at P12 indicated by the dashed line. Somatic instability in 6-month-old zQ175 mice was most pronounced in the striatum, brainstem, spinal cord and liver, and the repeat had contracted in the pituitary. Somatic CAG expansion was prevented in zQ175 mice in which Msh3 was absent, and contractions had led to a reduction in the CAG repeat mode. Heterozygosity for Msh3 led to a decrease in the extent of repeat expansion.