Prions, prionoids and pathogenic proteins in Alzheimer disease

Prion. 2013 Jan-Feb;7(1):55-9. doi: 10.4161/pri.23061. Epub 2012 Dec 3.

Abstract

Like patients with prion disease, Alzheimer patients suffer from a fatal, progressive form of dementia. There is growing evidence that amyloid-β (Aβ) aggregates may be transmissible similar to prions, at least under extreme experimental conditions. However, unlike mice infected with prion protein (PrP) prions, those inoculated with Aβ do not die. The transmission of Aβ and PrP thus differs conspicuously in the neurological effects they induce in their hosts, the difference being no less than a matter of life and death. Far from being a mere academic nuance, this distinction between Aβ and PrP begs the crucial questions of what, exactly, controls prion toxicity and how prion toxicity relates to prion infectivity.

Keywords: Alzheimer’s disease; PrP; amyloid-β; pathogenic proteins; prionoids; prions; tau.

Publication types

  • Review

MeSH terms

  • Alzheimer Disease / metabolism*
  • Alzheimer Disease / pathology*
  • Amyloid beta-Peptides / analysis
  • Amyloid beta-Peptides / metabolism*
  • Amyloid beta-Peptides / toxicity
  • Animals
  • Brain / metabolism
  • Brain / pathology
  • Humans
  • Prions / analysis
  • Prions / metabolism*
  • Prions / toxicity

Substances

  • Amyloid beta-Peptides
  • Prions