Walsh & Hoyt: Prion Diseases: The Future

Much remains to be learned about prion diseases. Transgenic mice have contributed invaluably to our understanding of these diseases and will continue to push the boundaries in this field. Undoubtedly, other prion diseases will be discovered, perhaps among other well-characterized and familiar neurodegenerative, myopathic, or neuropathic disorders. The concept of the prion as a benign host protein that undergoes a conformational change to produce a uniformly fatal disease may be applicable to other degenerative disorders such as Alzheimer disease, amyotrophic lateral sclerosis, or Parkinson disease. Alzheimer disease most notably is associated with amyloid plaques, which, although distinct from PrP plaques, show many similarities. Therapeutic options will also emerge, potentially exploiting the unique biochemical properties and as yet unknown mechanisms that underlie the neurotoxicity of PrPSc.