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. 2013 Sep 5;8(9):e72907. doi: 10.1371/journal.pone.0072907

Figure 5. Partial Rescue of EDM1 phenotype in skeletal muscle by over-expression of MBNL1.

Figure 5

A. RNA-IP using a monoclonal antibody against MBNL1 pulls down the GFP-DMPK 3′UTR mRNA in both the EDM1 and adult-onset DM1 mice but not mice expressing EGFP only. Beads, IgG and RT -ve controls were used to show MBNL1 specificity. EGFP was amplified to detect the transgene. B. MBNL1 was confirmed to be up-regulated by qRT-PCR and C. by western blot analysis in the MBNL1 treated leg but not the EGFP treated leg. D. Median myotonia score in EDM1 mice expressing MBNL1 and EGFP in their TA muscles shows a slight reduction in myotonia due the presence of MBNL1 but not EGFP. E. mRNA splicing was also partially improved for SmyD1. Clcn1-exon7a showed no change. Number of samples (n) provided along with average or median in the tables along with standard deviation. Two Sample T-Test was used to determine statistical significance with p-values displayed.