Fig_7.tif (1.6 MB)

Deregulation of the mitochondrial pathway in the OPMD mouse model.

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posted on 2015-03-27, 04:22 authored by Aymeric Chartier, Pierre Klein, Stéphanie Pierson, Nicolas Barbezier, Teresa Gidaro, François Casas, Steven Carberry, Paul Dowling, Laurie Maynadier, Maëlle Bellec, Martine Oloko, Claude Jardel, Bodo Moritz, George Dickson, Vincent Mouly, Kay Ohlendieck, Gillian Butler-Browne, Capucine Trollet, Martine Simonelig

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 Rplp0 mRNA. Means are from three biological replicates, error bars represent standard deviation. * p-value <0.05, ** p-value <0.01, *** p-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. RpL32 is a negative control mRNA encoding a ribosomal protein. Profiles of ePAT assays using the ImageJ software are shown.