Figure 6From: Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patientsA model for the involvement of UPS in OPMD disease pathology. In muscle, age-associated proteasome down-regulation affects expPABPN1 protein accumulation. Elevated expPABPN1 accumulation affects proteasome deregulation during disease onset. Expression profiles of E3-ligases can be sued to separate disease onset from progression.Back to article page