α-Synuclein Amyloids Hijack Prion Protein to Gain Cell Entry, Facilitate Cell-to-Cell Spreading and Block Prion Replication

Sci Rep. 2017 Aug 30;7(1):10050. doi: 10.1038/s41598-017-10236-x.

Abstract

The precise molecular mechanism of how misfolded α-synuclein (α-Syn) accumulates and spreads in synucleinopathies is still unknown. Here, we show the role of the cellular prion protein (PrPC) in mediating the uptake and the spread of recombinant α-Syn amyloids. The in vitro data revealed that the presence of PrPC fosters the higher uptake of α-Syn amyloid fibrils, which was also confirmed in vivo in wild type (Prnp +/+) compared to PrP knock-out (Prnp -/-) mice. Additionally, the presence of α-Syn amyloids blocked the replication of scrapie prions (PrPSc) in vitro and ex vivo, indicating a link between the two proteins. Indeed, whilst PrPC is mediating the internalization of α-Syn amyloids, PrPSc is not able to replicate in their presence. This observation has pathological relevance, since several reported case studies show that the accumulation of α-Syn amyloid deposits in Creutzfeldt-Jakob disease patients is accompanied by a longer disease course.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid / administration & dosage
  • Amyloid / genetics
  • Amyloid / metabolism*
  • Animals
  • Brain / metabolism
  • Brain / pathology*
  • Cell Line, Tumor
  • Creutzfeldt-Jakob Syndrome / genetics
  • Creutzfeldt-Jakob Syndrome / metabolism*
  • Creutzfeldt-Jakob Syndrome / pathology
  • Endopeptidase K / chemistry
  • Gene Expression Regulation
  • Humans
  • Injections, Intraventricular
  • Mice
  • Mice, Knockout
  • Neurons / metabolism*
  • Neurons / pathology
  • Prion Proteins / genetics
  • Prion Proteins / metabolism*
  • Protein Binding
  • Protein Transport
  • Recombinant Proteins / genetics
  • Recombinant Proteins / metabolism
  • Signal Transduction
  • Stereotaxic Techniques
  • Tyrosine 3-Monooxygenase / genetics
  • Tyrosine 3-Monooxygenase / metabolism
  • alpha-Synuclein / genetics
  • alpha-Synuclein / metabolism*

Substances

  • Amyloid
  • Prion Proteins
  • Prnp protein, mouse
  • Recombinant Proteins
  • alpha-Synuclein
  • Tyrosine 3-Monooxygenase
  • Endopeptidase K