Progressive changes in UPS gene expression. Progression trends for selected genes in mice (i) and humans (ii). Expression values were normalized to six-week-old WT in mice, and to young healthy controls (19 years old on average) in humans. P-values demonstrate the significance of differences in expression trends between controls and OPMD samples. A) The age-associated progression trend is indicated by P-value >0.05. B) The genotype-specific progression trend is indicated by P-values <0.05. SD represents variations in mice (6 weeks N = 5 and 26 weeks N = 6) and in humans (expPABPN1 carriers N = 4 and controls N = 5). C) RT Q-PCR validation of selected deregulated genes in UPS was carried out on skeletal muscles of 6-week-old and 26-week-old mice. Histograms show the log2(ratio) of the measured expression values using microarray and Q-PCR. Significant changes with the P <0.05 are indicated by *.