Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy
J. Clin. Invest. Guey-Shin Wang, et al. 117:2802 doi:10.1172/JCI32308 [
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Figure 1Generation of bitransgenic mouse expressing expanded CUG RNA in heart. (
A) EpA960 and EpA0 transgene constructs. The spliced mRNA transcripts from the nonrecombined (top) and recombined alleles (bottom) are indicated in blue above the gene diagrams. The mRNAs from the recombined EpA0 and EpA960 alleles are identical except for the presence or absence of the CUG repeats. Primer pairs used for RT-PCR analysis of nonrecombined and recombined alleles are shown. The CMV enhancer and β-actin promoter drive the transcription ubiquitously. Black boxes represent segments of different exons added for splicing. The cassette containing SV40 polyadenylation sites that prevent expression of RNA from downstream gene segments is located between 2
loxP sites. (
B) Relative levels of the nonrecombined transgene mRNAs in different tissues from EpA960 and EpA0 transgenic mice using real-time RT-PCR. Results are from 3 mice from each line, with the exception of the muscle sample, taken from 1 mouse in line EpA960/MCM 1332. (
C) Relative expression of EpA960(R) and EpA0(R) mRNAs from the recombined alleles after Cre-mediated recombination in heart.