Supporting Information

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Fig. 5. Genomic organization of shortstop transgene. Schematic describing the genomic organization of the shortstop truncated transgene, including primer positions for PCR in Fig. 1B.

Fig. 6. Comparison of shortstop, R6/2, and N171 genomic content. The genomic content of the truncated huntingtin mouse models including shortstop, R6/2, and N171 is shown. The reference sequence was human huntingtin accession no. NM_002111. (1)

1. Schilling, G., Becher, M. W., Sharp, A. H., Jinnah, H. A., Duan, K., Kotzuk, J. A., Slunt, H. H., Ratovitski, T., Cooper, J. K., Jenkins, N. A., et al. (1999) Hum. Mol. Genet. 8, 397-407.

Table 1. Nuclear localization of htt is accelerated in the shortstop (SS) mouse and exhibits regional differences compared with the full-length YAC128 mouse

Sections from YAC128 and shortstop mice at 3, 6, 9, 12, and 18 months of age were immunostained with EM48 to determine localization of htt to the nucleus. Regions were scored as -, no labeling; +, light labeling; ++, dark labeling.

Table 2. Huntingtin inclusion formation is accelerated and more widespread in the shortstop (SS) mouse compared with the full-length YAC128 mouse

Sections from YAC128 and shortstop mice at 3, 6, 9, 12, and 18 months of age were immunostained with EM48 for huntingtin inclusions. Regions were scored as -, no cells with inclusions; +, few cells with nuclear inclusions (1-5%); ++, scattered cells with nuclear inclusions (<50%); +++, numerous cells with nuclear inclusions (50-100%).