Neuropathogenesis of Prion Disease

Louise Davidson; Richard Knight


Future Neurology. 2014;9(2):135-147. 

In This Article

Future Perspective

Given the complexity of this area and the present uncertainties, it is difficult to predict future developments. However, it seems likely that the main areas of progress, over the next few years, will concern:

  • Further understanding of the PrP misfolding mechanisms with clearer identification of oligomeric intermediates and their role in neurotoxicity;

  • Further understanding of interactions between identified toxic species and normal PrPC leading to clarification of the role of PrPC in neuropathogenesis;

  • Increasing evidence of important interactions between PrP and other proteins that may enhance our overall understanding of neurodegenerative diseases and not just prion diseases;

  • Further study of the recently reported work on the potential role of the unfolded protein response pathway may shed more light on neuropathogenesis of neurodegenerative diseases (including prion diseases), as well as improving understanding of the relationship between PrPC and PrPSc.

Whether this increased understanding will translate into useful therapies over the same time scale is very uncertain.