A conformational switch controlling the toxicity of the prion protein.


Journal

Nature structural & molecular biology
ISSN: 1545-9985
Titre abrégé: Nat Struct Mol Biol
Pays: United States
ID NLM: 101186374

Informations de publication

Date de publication:
08 2022
Historique:
received: 01 12 2021
accepted: 06 07 2022
pubmed: 11 8 2022
medline: 16 8 2022
entrez: 10 8 2022
Statut: ppublish

Résumé

Prion infections cause conformational changes of the cellular prion protein (PrP

Identifiants

pubmed: 35948768
doi: 10.1038/s41594-022-00814-7
pii: 10.1038/s41594-022-00814-7
pmc: PMC9371974
doi:

Substances chimiques

Antibodies 0
Ligands 0
PrPC Proteins 0
Prion Proteins 0
Prions 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

831-840

Subventions

Organisme : Biotechnology and Biological Sciences Research Council
Pays : United Kingdom

Informations de copyright

© 2022. The Author(s).

Références

Brandner, S. et al. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379, 339–343 (1996).
doi: 10.1038/379339a0
Lakkaraju, A. K. K. et al. Loss of PIKfyve drives the spongiform degeneration in prion diseases. EMBO Mol. Med. 13, e14714 (2021).
McNally, K. L., Ward, A. E. & Priola, S. A. Cells expressing anchorless prion protein are resistant to scrapie infection. J. Virol. 83, 4469–4475 (2009).
doi: 10.1128/JVI.02412-08
Wulf, M. A., Senatore, A. & Aguzzi, A. The biological function of the cellular prion protein: an update. BMC Biol. 15, 34 (2017).
doi: 10.1186/s12915-017-0375-5
Bueler, H. et al. Mice devoid of PrP are resistant to scrapie. Cell 73, 1339–1347 (1993).
doi: 10.1016/0092-8674(93)90360-3
Heppner, F. L. et al. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science 294, 178–182 (2001).
doi: 10.1126/science.1063093
Sonati, T. et al. The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein. Nature 501, 102–106 (2013).
doi: 10.1038/nature12402
Herrmann, U. S. et al. Prion infections and anti-PrP antibodies trigger converging neurotoxic pathways. PLoS Pathog. 11, e1004662 (2015).
doi: 10.1371/journal.ppat.1004662
Reimann, R. R. et al. Differential toxicity of antibodies to the prion protein. PLoS Pathog. 12, e1005401 (2016).
doi: 10.1371/journal.ppat.1005401
Frontzek, K. et al. Neurotoxic antibodies against the prion protein do not trigger prion replication. PLoS ONE 11, e0163601 (2016).
doi: 10.1371/journal.pone.0163601
Bardelli, M. et al. A bispecific immunotweezer prevents soluble PrP oligomers and abolishes prion toxicity. PLoS Pathog. 14, e1007335 (2018).
doi: 10.1371/journal.ppat.1007335
Baral, P. K. et al. Structural studies on the folded domain of the human prion protein bound to the Fab fragment of the antibody POM1. Acta Crystallogr. Sect. D Biol. Crystallogr. 68, 1501–1512 (2012).
doi: 10.1107/S0907444912037328
Mahal, S. P. et al. Prion strain discrimination in cell culture: the cell panel assay. Proc. Natl Acad. Sci. USA 104, 20908–20913 (2007).
doi: 10.1073/pnas.0710054104
Falsig, J. et al. A versatile prion replication assay in organotypic brain slices. Nat. Neurosci. 11, 109–117 (2008).
doi: 10.1038/nn2028
Nuvolone, M. et al. Strictly co-isogenic C57BL/6J-Prnp
doi: 10.1084/jem.20151610
Fischer, M. et al. Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie. EMBO J. 15, 1255–1264 (1996).
doi: 10.1002/j.1460-2075.1996.tb00467.x
Perrier, V. et al. Anti-PrP antibodies block PrPSc replication in prion-infected cell cultures by accelerating PrPC degradation. J. Neurochem. 89, 454–463 (2004).
doi: 10.1111/j.1471-4159.2004.02356.x
White, A. R. et al. Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature 422, 80–83 (2003).
doi: 10.1038/nature01457
Simonelli, L. et al. Mapping antibody epitopes by solution NMR spectroscopy: practical considerations. Methods Mol. Biol. 1785, 29–51 (2018).
doi: 10.1007/978-1-4939-7841-0_3
Wang, J. et al. A human bi-specific antibody against Zika virus with high therapeutic potential. Cell 171, 229–241(2017).
doi: 10.1016/j.cell.2017.09.002
Lakkaraju, A. K. et al. Loss of PIKfyve drives the spongiform degeneration in prion diseases. EMBO Mol. Med. 13, e14714 (2021).
doi: 10.15252/emmm.202114714
Brody, A. H. & Strittmatter, S. M. Synaptotoxic signaling by amyloid beta oligomers in Alzheimer’s disease through prion protein and mGluR5. Adv. Pharm. 82, 293–323 (2018).
doi: 10.1016/bs.apha.2017.09.007
Rincon, M. Y. et al. Widespread transduction of astrocytes and neurons in the mouse central nervous system after systemic delivery of a self-complementary AAV-PHP.B vector. Gene Ther. 25, 83–92 (2018).
doi: 10.1038/s41434-018-0005-z
Herrmann, U. S. et al. Structure-based drug design identifies polythiophenes as antiprion compounds. Sci. Transl. Med. 7, 299ra123 (2015).
doi: 10.1126/scitranslmed.aab1923
Polymenidou, M. et al. The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes. PLoS One 3, e3872 (2008).
doi: 10.1371/journal.pone.0003872
Ballmer, B. A. et al. Modifiers of prion protein biogenesis and recycling identified by a highly parallel endocytosis kinetics assay. J. Biol. Chem. 292, 8356–8368 (2017).
doi: 10.1074/jbc.M116.773283
Yang, J. et al. The I-TASSER Suite: protein structure and function prediction. Nat. Methods 12, 7–8 (2015).
doi: 10.1038/nmeth.3213
Berendsen, H. J. C., van der Spoel, D. & van Drunen, R. GROMACS: a message-passing parallel molecular dynamics implementation. Comput. Phys. Commun. 91, 43–56 (1995).
doi: 10.1016/0010-4655(95)00042-E
Hornemann, S., von Schroetter, C., Damberger, F. F. & Wuthrich, K. Prion protein-detergent micelle interactions studied by NMR in solution. J. Biol. Chem. 284, 22713–22721 (2009).
doi: 10.1074/jbc.M109.000430
Hornemann, S., Christen, B., von Schroetter, C., Perez, D. R. & Wuthrich, K. Prion protein library of recombinant constructs for structural biology. FEBS J. 276, 2359–2367 (2009).
doi: 10.1111/j.1742-4658.2009.06968.x
Zahn, R., von Schroetter, C. & Wuthrich, K. Human prion proteins expressed in Escherichia coli and purified by high-affinity column refolding. FEBS Lett. 417, 400–404 (1997).
doi: 10.1016/S0014-5793(97)01330-6
Frontzek, K. et al. Autoantibodies against the prion protein in individuals with PRNP mutations. Neurology 2020, 1–10 (2020).
Hussain, R. et al. CDApps: integrated software for experimental planning and data processing at beamline B23, Diamond Light Source. Corrigendum. J. Synchrotron Radiat. 22, 862 (2015).
doi: 10.1107/S1600577515007602
Provencher, S. W. & Glockner, J. Estimation of globular protein secondary structure from circular dichroism. Biochemistry 20, 33–37 (1981).
doi: 10.1021/bi00504a006

Auteurs

Karl Frontzek (K)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Marco Bardelli (M)

Institute for Research in Biomedicine, Università della Svizzera italiana, Bellinzona, Switzerland.
PetMedix Ltd, Babraham Research Campus, Cambridge, UK.

Assunta Senatore (A)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Anna Henzi (A)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Regina R Reimann (RR)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Seden Bedir (S)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Marika Marino (M)

Laboratory of Glia Biology, VIB-KU Leuven Center for Brain and Disease Research, Leuven, Belgium.

Rohanah Hussain (R)

B23 Beamline, Diamond Light Source, Harwell Science Innovation Campus, Didcot, UK.

Simon Jurt (S)

University of Zurich, Department of Chemistry, Zurich, Switzerland.

Georg Meisl (G)

Department of Chemistry, University of Cambridge, Cambridge, UK.

Mattia Pedotti (M)

Institute for Research in Biomedicine, Università della Svizzera italiana, Bellinzona, Switzerland.

Federica Mazzola (F)

Institute for Research in Biomedicine, Università della Svizzera italiana, Bellinzona, Switzerland.

Giuliano Siligardi (G)

B23 Beamline, Diamond Light Source, Harwell Science Innovation Campus, Didcot, UK.

Oliver Zerbe (O)

University of Zurich, Department of Chemistry, Zurich, Switzerland.

Marco Losa (M)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Tuomas Knowles (T)

Department of Chemistry, University of Cambridge, Cambridge, UK.

Asvin Lakkaraju (A)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Caihong Zhu (C)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Petra Schwarz (P)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Simone Hornemann (S)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland.

Matthew G Holt (MG)

Laboratory of Glia Biology, VIB-KU Leuven Center for Brain and Disease Research, Leuven, Belgium.
Laboratory of Synapse Biology, Instituto de Investigação e Inovação em Saúde (i3S), University of Porto, Porto, Portugal.

Luca Simonelli (L)

Institute for Research in Biomedicine, Università della Svizzera italiana, Bellinzona, Switzerland.

Luca Varani (L)

Institute for Research in Biomedicine, Università della Svizzera italiana, Bellinzona, Switzerland. luca.varani@irb.usi.ch.

Adriano Aguzzi (A)

Institute of Neuropathology, University of Zurich, Zurich, Switzerland. adriano.aguzzi@usz.ch.

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