10.1371/journal.pgen.1005092.g007 Aymeric Chartier Aymeric Chartier Pierre Klein Pierre Klein Stéphanie Pierson Stéphanie Pierson Nicolas Barbezier Nicolas Barbezier Teresa Gidaro Teresa Gidaro François Casas François Casas Steven Carberry Steven Carberry Paul Dowling Paul Dowling Laurie Maynadier Laurie Maynadier Maëlle Bellec Maëlle Bellec Martine Oloko Martine Oloko Claude Jardel Claude Jardel Bodo Moritz Bodo Moritz George Dickson George Dickson Vincent Mouly Vincent Mouly Kay Ohlendieck Kay Ohlendieck Gillian Butler-Browne Gillian Butler-Browne Capucine Trollet Capucine Trollet Martine Simonelig Martine Simonelig Deregulation of the mitochondrial pathway in the OPMD mouse model. Public Library of Science 2015 ccr mRNAs encoding mitochondrial proteins cleavage poly Drosophila OPMD model uncovers deadenylation regulator Smaug pabpn candidate genes encoding RNA binding proteins role Oculopharyngeal Muscular Dystrophy Pathogenesis Oculopharyngeal mitochondrial dysfunction 2015-03-27 04:22:30 Figure https://plos.figshare.com/articles/figure/_Deregulation_of_the_mitochondrial_pathway_in_the_OPMD_mouse_model_/1359255 <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>