Amyloid aggregates of the HET-s prion protein are infectious

Proc Natl Acad Sci U S A. 2002 May 28;99(11):7402-7. doi: 10.1073/pnas.072199199.

Abstract

The [Het-s] infectious element of the filamentous fungus Podospora anserina is a prion. We have recently reported that recombinant HET-s protein aggregates in vitro into amyloid fibers. In vivo, the protein aggregates specifically in the [Het-s] prion strains. Here, we show that biolistic introduction of aggregated recombinant HET-s protein into fungal cells induces emergence of the [Het-s] prion with a high frequency. Thus, we demonstrate that prion infectivity can be created de novo, in vitro from recombinant protein in this system. Although the amyloid filaments formed from HET-s could transmit [Het-s] efficiently, neither the soluble form of the protein nor amorphous aggregates would do so. In addition, we have found that (i) [Het-s] infectivity correlates with the ability to convert HET-s to amyloids in vitro, (ii) [Het-s] infectivity is resistant to proteinase K digestion, and (iii) HET-s aggregates formed in vivo in [Het-s] strains have the ability to convert the recombinant protein to aggregates. Together, our data designate the HET-s amyloids as the molecular basis of [Het-s] prion propagation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloid / chemistry
  • Amyloid / metabolism*
  • Biolistics
  • Endopeptidase K
  • Escherichia coli / genetics
  • Fungal Proteins / chemistry
  • Fungal Proteins / isolation & purification
  • Fungal Proteins / metabolism*
  • Prions / metabolism*
  • Prions / pathogenicity
  • Recombinant Proteins / chemistry
  • Recombinant Proteins / metabolism
  • Sordariales / growth & development
  • Sordariales / pathogenicity
  • Sordariales / physiology*

Substances

  • Amyloid
  • Fungal Proteins
  • HET-S protein, Podospora anserina
  • Prions
  • Recombinant Proteins
  • Endopeptidase K