Activation of p53-regulated pro-apoptotic signaling pathways in PrP-mediated myopathy.

ABSTRACT:

Background

We have reported that doxycycline-induced over-expression of wild type prion protein (PrP) in skeletal muscles of Tg(HQK) mice is sufficient to cause a primary myopathy with no signs of peripheral neuropathy. The preferential accumulation of the truncated PrP C1 fragment was closely correlated with these myopathic changes. In this study we use gene expression profiling to explore the temporal program of molecular changes underlying the PrP-mediated myopathy.

Results

We used DNA microarrays, and confirmatory real-time PCR and Western blot analysis to demonstrate deregulation of a large number of genes in the course of the progressive myopathy in the skeletal muscles of doxycycline-treated Tg(HQK) mice. These include the down-regulation of genes coding for the myofi

SUBMITTER: Liang J 

PROVIDER: S-EPMC2683871 | biostudies-literature | 2009 Apr

REPOSITORIES: biostudies-literature