Deregulation of the mitochondrial pathway in the OPMD mouse model.

<p>A) Number of deregulated genes in A17.1 mouse skeletal muscles using microarrays. T1, 6 weeks; T2, 18 weeks; T3, 26 weeks. B) Venn diagram of overlapping down-regulated genes at T1, T2 and T3. C) Number of nuclear genes encoding mitochondrial respiratory chain complex subunits down-regulated in A17.1 mouse muscles. D) Quantification of levels of mRNAs encoding mitochondrial respiratory chain subunits in control (WT) and A17.1 quadriceps skeletal muscle at T1, using RT-qPCR. Normalization was with <i>Rplp0</i> mRNA. Means are from three biological replicates, error bars represent standard deviation. * <i>p</i>-value <0.05, ** <i>p</i>-value <0.01, *** <i>p</i>-value <0.001, using the Student’s t-Test. E) ePAT assays of mRNAs encoding mitochondrial proteins in control (WT) and A17.1 quadriceps skeletal muscles. Arrows indicate poly(A) tails of 12A. Accumulation of 12A poly(A) tails was visible in A17.1 muscles at T2 and/or T3. <i>RpL32</i> is a negative control mRNA encoding a ribosomal protein. Profiles of ePAT assays using the ImageJ software are shown.</p>