Figure 7
From: Activation of p53-regulated pro-apoptotic signaling pathways in PrP-mediated myopathy
Mechanism of PrP-mediated myopathy. Accumulation of an N-terminal truncated PrP C1 fragment in muscle activates p53 resulting in the induction of p53-regulated pro-apoptotic networks and myopathic changes. PrPC over-expression also results in down-regulation of MEF2C, which may be partially responsible for the progressive central nuclei localization observed in the muscles of Dox-treated Tg(HQK) mice.