Figure 1.

Upregulation of Cpne2 and UchL1 protein, and absence of short poly(CUG) RNA, in mice that express CUG^exp RNA. (A) Specificity of anti-Cpne2 antibody. Immunoblot shows detection of appropriate 60 kDa protein in COS cells transfected with Cpne2 expression construct, but not in mock transfected cells. (B) Cpne2 protein is detected by immunoblot in muscle homogenate from HSA^LR20b and HSA^LR41 mice, but not in muscle from wild-type (WT) mice of the same strain background (FVB). Gapdh serves as loading control. A parallel blot was probed with the anti-UchL1 antibody. (C) RNA blot analysis was performed under conditions designed to detect short CUG repeats. Shown are RNAs isolated from quadriceps muscle of three HSA^LR transgenic mice and WT control (FVB). Probes for miR-1 and miR-133 RNAs serve as controls for RNA loading and miRNA detection. Note that short poly(CUG) RNA was undetectable even after a 20-fold longer film exposure (not shown).