The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein

@article{Sonati2013TheTO,
  title={The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein},
  author={Tiziana Sonati and Regina Rose Reimann and Jeppe Falsig and Pravas Kumar Baral and Tracy O'Connor and Simone Hornemann and Sine Yaganoglu and Bei Li and Uli S. Herrmann and Barbara Wieland and Mridula Swayampakula and Muhammad Hafizur Rahman and Dipankar Das and Nat N V Kav and Roland Riek and Pawel P. Liberski and Michael N. G. James and Adriano Aguzzi},
  journal={Nature},
  year={2013},
  volume={501},
  pages={102-106}
}
Prion infections cause lethal neurodegeneration. This process requires the cellular prion protein (PrPC; ref. 1), which contains a globular domain hinged to a long amino-proximal flexible tail. Here we describe rapid neurotoxicity in mice and cerebellar organotypic cultured slices exposed to ligands targeting the α1 and α3 helices of the PrPC globular domain. Ligands included seven distinct monoclonal antibodies, monovalent Fab1 fragments and recombinant single-chain variable fragment… CONTINUE READING
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