MSH3 and juvenile Huntington disease: We also considered the 266 genes that comprise the ‘striatal signature’ of robustly dysregulated genes across multiple Huntington's disease models.51 Of these, 258 were dysregulated in the zQ175 striatum and a reversal of expression toward wild-type levels in the absence of MSH3 was not observed for any of these genes (Fig. 5E).