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@ARTICLE{Lers:137547,
      author       = {Lüers, and Bannach, Oliver and Stöhr, Jan and Wördehoff,
                      Michael Marius and Wolff, Martin and Nagel-Steger, Luitgard
                      and Riesner, Detlev and Willbold, Dieter and Birkmann, Eva},
      title        = {{S}eeded {F}ibrillation as {M}olecular {B}asis of the
                      {S}pecies {B}arrier in {H}uman {P}rion {D}iseases},
      journal      = {PLoS one},
      volume       = {8},
      number       = {8},
      issn         = {1932-6203},
      address      = {Lawrence, Kan.},
      publisher    = {PLoS},
      reportid     = {FZJ-2013-03980},
      pages        = {e72623 -},
      year         = {2013},
      abstract     = {Prion diseases are transmissible spongiform
                      encephalopathies in humans and animals, including scrapie in
                      sheep, bovine spongiform encephalopathy (BSE) in cattle,
                      chronic wasting disease (CWD) in deer, and Creutzfeldt-Jakob
                      disease (CJD) in humans. The hallmark of prion diseases is
                      the conversion of the host-encoded prion protein (PrPC) to
                      its pathological isoform PrPSc, which is accompanied by PrP
                      fibrillation. Transmission is not restricted within one
                      species, but can also occur between species. In some cases a
                      species barrier can be observed that results in limited or
                      unsuccessful transmission. The mechanism behind interspecies
                      transmissibility or species barriers is not completely
                      understood. To analyse this process at a molecular level, we
                      previously established an in vitro fibrillation assay, in
                      which recombinant PrP (recPrP) as substrate can be
                      specifically seeded by PrPSc as seed. Seeding with purified
                      components, with no additional cellular components, is a
                      direct consequence of the “prion-protein-only”
                      hypothesis. We therefore hypothesise, that the species
                      barrier is based on the interaction of PrPC and PrPSc.
                      Whereas in our earlier studies, the interspecies
                      transmission in animal systems was analysed, the focus of
                      this study lies on the transmission from animals to humans.
                      We therefore combined seeds from species cattle, sheep and
                      deer (BSE, scrapie, CWD) with human recPrP. Homologous
                      seeding served as a control. Our results are consistent with
                      epidemiology, other in vitro aggregation studies, and
                      bioassays investigating the transmission between humans,
                      cattle, sheep, and deer. In contrast to CJD and BSE seeds,
                      which show a seeding activity we can demonstrate a species
                      barrier for seeds from scrapie and CWD in vitro. We could
                      show that the seeding activity and therewith the molecular
                      interaction of PrP as substrate and PrPSc as seed is
                      sufficient to explain the phenomenon of species barriers.
                      Therefore our data supports the hypothesis that CWD is not
                      transmissible to humans.},
      cin          = {ICS-6},
      ddc          = {500},
      cid          = {I:(DE-Juel1)ICS-6-20110106},
      pnm          = {452 - Structural Biology (POF2-452)},
      pid          = {G:(DE-HGF)POF2-452},
      typ          = {PUB:(DE-HGF)16},
      UT           = {WOS:000324527300092},
      pubmed       = {pmid:23977331},
      doi          = {10.1371/journal.pone.0072623},
      url          = {https://juser.fz-juelich.de/record/137547},
}