Figure 1

Characterization of HD monkey and HD monkey skin fibroblasts. (A) The presence of transgenes "mutant htt and GFP" in brain and peripheral tissues of HD monkey was confirmed by PCR analysis using primer sets specifically for mutant htt (top panel) and for the GFP gene (bottom panel). (B) Expression of the transgenic mutant HTT was confirmed by Western blot analysis in brain and peripheral tissues using mEM48 (top panel). Immunoblot revealed high-molecular-mass oligomeric HTT (arrow). The blot was also probed with an antibody to γ-tubulin as an internal control (bottom panel)., Wild-type (WT) non-transgenic monkey. (C) Immunostaining of primary cultured skin fibroblast of transgenic HD monkey using mEM48 demonstrated that transgenic mutant htt was distributed in the nuclei (arrow; C-c) and intranuclear inclusions (arrowheads; C-c) were also revealed. Expression of GFP was also revealed by epifluorescent microscopy (d). (C-a) transmission light image; (C-b) Hoechst DNA staining; (C-c) mEM48 staining; (C-d) epifluorescent light image of GFP; (C-e) overlay image. Scale bar = 5 μm.