Figure 5From: Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patientsThe effect of altered proteasome activity on expPABPN1 accumulation and aggregation. A) Substantial deregulation of proteasome and immunoproteasome encoding genes in mice and humans. Down-regulation (green) is more pronounced in the core subunit of the proteasome. Immunoproteasome shows consistent up-regulation (red) in both organisms. B) Western blot analysis of YFP-Ala16-PABPN1 transfected C2C12 cells that were treated with 5 μM MG132 or 5 nM PR619. Control cells were treated with DMSO. C) Images show YFP-Ala16-PABPN1 localization in C2C12 after mock-treatment (DMSO), 5 μM MG132 or 5 U/ml IFNγ. Scale bar equals 10 μm. Histograms show the integrated intensity of YFP-Ala16-PABPN1 (i) or Histone4-CFP (control) (ii), and the percentage of cells with INI in YFP-Ala16-PABPN1 expressing cells (iii). Averages represent 509, 773 and 476 cells for DMSO, MG132 and IFNγ, respectively. Significant difference between treatments is reflected by P-values.Back to article page