The buildup of prion protein as it replicates induces "persistent translational repression" of general protein synthesis by eIF2alpha, which in turn was associated with synaptic failure and cell death. According to NEUROLOGY TODAY, the normally protective mechanism is over-activated with fatal prolonged failure of protein synthesis.
Researchers then used viral vectors to overexpress an enzyme specific eIF2alpha-P phosphatase called GADD34 to reduce prion protein levels, protecting brain hippocampal cells which are commonly targeted in the sick with memory disorders.
Researchers are now looking for drugs that could work on this pathway in humans to provide a unified treatment approach for these incurable diseases.
Nature 2012;E-pub 2012 May 6