We also found many differentially expressed genes related to the Huntington disease (HD) pathway in both areas of the MPS II mouse model; among these, Bbc3, Bdnf, Crebbp, Dctn1, Dlg4, Gpx1, Grin1, Grin2b, Itpr1, and Pparg are up-regulated, while Dnaic2, Dnali1, Hap1, and Vdac2 are down-regulated. This evidence concerns the gene ITPR1 and juvenile Huntington disease.