Figure 2.  Alternative splicing of a known CELF target is rescued in bitransgenic HSA ^LR / Myo -CELF∆ mice.  Nrap exon 12 skipping is induced in the thigh muscle of expanded CUG repeat RNA-expressing HSA^LR mice, but is normal in bitransgenic HSA^LR/Myo-CELF∆ transgenic mice that also express a dominant negative CELF protein.  (A) A representative RT-PCR gel showing Nrap exon 12 included and exon skipped products in thigh muscle total RNA samples from wild type, transgenic, and bitransgenic mice.  (B) Quantitation of the extent of exon inclusion and exon skipping in thigh muscle samples from multiple individuals (n = 6 for wild type, n = 5 for HSA^LR, and n = 6 for HSA^LR/Myo-CELF∆).