Figure 3From: Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patientsProgressive 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 *.Back to article page