Mouse models for studying the formation and propagation of prions

J Biol Chem. 2014 Jul 18;289(29):19841-9. doi: 10.1074/jbc.R114.550707. Epub 2014 May 23.

Abstract

Prions are self-propagating protein conformers that cause a variety of neurodegenerative disorders in humans and animals. Mouse models have played key roles in deciphering the biology of prions and in assessing candidate therapeutics. The development of transgenic mice that form prions spontaneously in the brain has advanced our understanding of sporadic and genetic prion diseases. Furthermore, the realization that many proteins can become prions has necessitated the development of mouse models for assessing the potential transmissibility of common neurodegenerative diseases. As the universe of prion diseases continues to expand, mouse models will remain crucial for interrogating these devastating illnesses.

Keywords: Animal Model; Neurodegenerative Disease; Pathology; Prion; Protein Aggregation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Alzheimer Disease / etiology
  • Amyloid beta-Peptides / chemistry
  • Amyloid beta-Peptides / genetics
  • Amyloid beta-Peptides / metabolism
  • Animals
  • Brain / metabolism
  • Brain / pathology
  • Disease Models, Animal
  • Humans
  • Mice
  • Mice, Transgenic
  • Mutation
  • Parkinson Disease / etiology
  • Prion Diseases / etiology*
  • Prion Diseases / metabolism
  • Prion Diseases / therapy
  • Prions / chemistry
  • Prions / genetics
  • Prions / metabolism*
  • alpha-Synuclein / chemistry
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism
  • tau Proteins / chemistry
  • tau Proteins / genetics
  • tau Proteins / metabolism

Substances

  • Amyloid beta-Peptides
  • Prions
  • alpha-Synuclein
  • tau Proteins