Sequence-dependent prion protein misfolding and neurotoxicity

J Biol Chem. 2010 Nov 19;285(47):36897-908. doi: 10.1074/jbc.M110.174391. Epub 2010 Sep 3.

Abstract

Prion diseases are neurodegenerative disorders caused by misfolding of the normal prion protein (PrP) into a pathogenic "scrapie" conformation. To better understand the cellular and molecular mechanisms that govern the conformational changes (conversion) of PrP, we compared the dynamics of PrP from mammals susceptible (hamster and mouse) and resistant (rabbit) to prion diseases in transgenic flies. We recently showed that hamster PrP induces spongiform degeneration and accumulates into highly aggregated, scrapie-like conformers in transgenic flies. We show now that rabbit PrP does not induce spongiform degeneration and does not convert into scrapie-like conformers. Surprisingly, mouse PrP induces weak neurodegeneration and accumulates small amounts of scrapie-like conformers. Thus, the expression of three highly conserved mammalian prion proteins in transgenic flies uncovered prominent differences in their conformational dynamics. How these properties are encoded in the amino acid sequence remains to be elucidated.

Publication types

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

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Animals, Genetically Modified
  • Blotting, Western
  • Chromatography, Gel
  • Conserved Sequence
  • Cricetinae
  • Drosophila melanogaster / genetics*
  • Drosophila melanogaster / growth & development
  • Drosophila melanogaster / metabolism
  • Female
  • Fluorescent Antibody Technique
  • Immunoprecipitation
  • Locomotion / physiology
  • Male
  • Mice
  • Molecular Sequence Data
  • Neurotoxicity Syndromes / etiology*
  • Neurotoxicity Syndromes / metabolism
  • Neurotoxicity Syndromes / pathology
  • Prion Diseases / genetics
  • Prion Diseases / metabolism
  • Prion Diseases / pathology*
  • Prions / chemistry*
  • Prions / genetics*
  • Prions / metabolism
  • Protein Conformation
  • Protein Folding*
  • RNA, Messenger / genetics
  • Rabbits
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Homology, Amino Acid

Substances

  • Prions
  • RNA, Messenger