Cellular toxicity of scrapie prions in prion diseases; a biochemical and molecular overview

Mol Biol Rep. 2023 Feb;50(2):1743-1752. doi: 10.1007/s11033-022-07806-2. Epub 2022 Nov 29.

Abstract

Transmissible spongiform encephalopathies (TSEs) or prion diseases consist of a broad range of fatal neurological disorders affecting humans and animals. Contrary to Watson and Crick's 'central dogma', prion diseases are caused by a protein, devoid of DNA involvement. Herein, we briefly review various cellular and biological aspects of prions and prion pathogenesis focusing mainly on historical milestones, biosynthesis, degradation, structure-function of cellular and scrapie forms of prions .

Keywords: Cell death, Signal; Neurotoxicity; Prion protein; Protein biochemistry; Protein quality control; Transduction, Protein degradation.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Prion Diseases* / genetics
  • Prion Diseases* / metabolism
  • Prion Diseases* / pathology
  • Prions* / genetics
  • Scrapie* / genetics
  • Scrapie* / metabolism
  • Scrapie* / pathology
  • Sheep

Substances

  • Prions