Function and malfunction of the prion protein

Project Information

PRION2020

Grant agreement ID: 670958

DOI

10.3030/670958

Project closed

EC signature date 5 October 2015

Start date 1 November 2015

End date 31 October 2020

Funded under

• EXCELLENT SCIENCE - European Research Council (ERC)

Total cost

€ 2 500 000,00

EU contribution

€ 2 500 000,00

Coordinated by

UNIVERSITAT ZURICH
Switzerland

CORDIS provides links to public deliverables and publications of HORIZON projects.

Links to deliverables and publications from FP7 projects, as well as links to some specific result types such as dataset and software, are dynamically retrieved from OpenAIRE .

Publications

Peer reviewed articles (31)

Prion infection, transmission, and cytopathology modeled in a low-biohazard human cell line

Author(s): Merve Avar, Daniel Heinzer, Nicolas Steinke, Berre Doğançay, Rita Moos, Severine Lugan, Claudia Cosenza, Simone Hornemann, Olivier Andréoletti, Adriano Aguzzi
Published in: Life Science Alliance, Issue 3/8, 2020, Page(s) e202000814, ISSN 2575-1077
Publisher: Life Science Alliance
DOI: 10.26508/lsa.202000814

Loss of PIKfyve drives the spongiform degeneration in prion diseases

Author(s): Asvin K K Lakkaraju, Karl Frontzek, Emina Lemes, Uli Herrmann, Marco Losa, Rajlakshmi Marpakwar, Adriano Aguzzi
Published in: EMBO Molecular Medicine, Issue 13/9, 2021, ISSN 1757-4676
Publisher: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.202114714

The role of macrophage scavenger receptor 1 (Msr1) in prion pathogenesis

Author(s): Bei Li, Meiling Chen, Adriano Aguzzi, Caihong Zhu
Published in: Journal of Molecular Medicine, Issue 99/6, 2021, Page(s) 877-887, ISSN 0946-2716
Publisher: Springer Verlag
DOI: 10.1007/s00109-021-02061-7

LAG3 is not expressed in human and murine neurons and does not modulate α‐synucleinopathies

Author(s): Marc Emmenegger, Elena De Cecco, Marian Hruska‐Plochan, Timo Eninger, Matthias M Schneider, Melanie Barth, Elena Tantardini, Pierre de Rossi, Mehtap Bacioglu, Rebekah G Langston, Alice Kaganovich, Nora Bengoa‐Vergniory, Andrès Gonzalez‐Guerra, Merve Avar, Daniel Heinzer, Regina Reimann, Lisa M Häsler, Therese W Herling, Naunehal S Matharu, Natalie Landeck, Kelvin Luk, Ronald Melki, Philipp
Published in: EMBO Molecular Medicine, Issue 13/9, 2021, ISSN 1757-4676
Publisher: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.202114745

Protective anti‐prion antibodies in human immunoglobulin repertoires

Author(s): Assunta Senatore, Karl Frontzek, Marc Emmenegger, Andra Chincisan, Marco Losa, Regina Reimann, Geraldine Horny, Jingjing Guo, Sylvie Fels, Silvia Sorce, Caihong Zhu, Nathalie George, Stefan Ewert, Thomas Pietzonka, Simone Hornemann, Adriano Aguzzi
Published in: EMBO Molecular Medicine, Issue e12739, 2020, Page(s) 1-19, ISSN 1757-4676
Publisher: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.202012739

An integrated genomic approach to dissect the genetic landscape regulating the cell-to-cell transfer of α-synuclein

Author(s): Eleanna Kara, Alessandro Crimi, Anne Wiedmer, Marc Emmenegger, Claudia Manzoni, Sara Bandres-Ciga, Karishma D’Sa, Regina H. Reynolds, Juan A. Botía, Marco Losa, Veronika Lysenko, Manfredi Carta, Daniel Heinzer, Merve Avar, Andra Chincisan, Cornelis Blauwendraat, Sonia García-Ruiz, Daniel Pease, Lorene Mottier, Alessandra Carrella, Dezirae Beck-Schneider, Andreia D. Magalhães, Caroline Aemiseg
Published in: Cell Reports, Issue 35/10, 2021, Page(s) 109189, ISSN 2211-1247
Publisher: Cell Press
DOI: 10.1016/j.celrep.2021.109189

Toward Therapy of Human Prion Diseases

Author(s): Adriano Aguzzi, Asvin K.K. Lakkaraju, Karl Frontzek
Published in: Annual Review of Pharmacology and Toxicology, Issue 58/1, 2018, Page(s) 331-351, ISSN 0362-1642
Publisher: Annual Reviews, Inc.
DOI: 10.1146/annurev-pharmtox-010617-052745

Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity

Author(s): Despoina Goniotaki, Asvin K. K. Lakkaraju, Amulya N. Shrivastava, Pamela Bakirci, Silvia Sorce, Assunta Senatore, Rajlakshmi Marpakwar, Simone Hornemann, Fabrizio Gasparini, Antoine Triller, Adriano Aguzzi
Published in: PLOS Pathogens, Issue 13/11, 2017, Page(s) e1006733, ISSN 1553-7374
Publisher: PLOS Pathogens
DOI: 10.1371/journal.ppat.1006733

The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6

Author(s): Küffer, Alexander; Lakkaraju, Asvin K. K.; Mogha, Amit; Petersen, Sarah C.; Airich, Kristina; Doucerain, Cédric; Marpakwar, Rajlakshmi; Bakirci, Pamela; Senatore, Assunta; Monnard, Arnaud; Schiavi, Carmen; Nuvolone, Mario; Grosshans, Bianka; Hornemann, Simone; Bassilana, Frederic; Monk, Kelly R.; Aguzzi, Adriano
Published in: Nature, Issue 4, 2016, ISSN 0028-0836
Publisher: Nature Publishing Group
DOI: 10.5167/uzh-125485

Modifiers of prion protein biogenesis and recycling identified by a highly parallel endocytosis kinetics assay

Author(s): Ballmer, Boris A.; Moos, Rita; Liberali, Prisca; Pelkmans, Lucas; Hornemann, Simone; Aguzzi, Adriano
Published in: Journal of Biological Chemistry, Issue 292(20), 2017, Page(s) 8356-8368., ISSN 0021-9258
Publisher: American Society for Biochemistry and Molecular Biology Inc.
DOI: 10.5167/uzh-136555

Absolute Quantification of Amyloid Propagons by Digital Microfluidics

Author(s): Pfammatter, Manuela; Andreasen, Maria; Meisl, Georg; Taylor, Christopher G; Adamcik, Jozef; Bolisetty, Sreenath; Sanchez-Ferrer, Antoni; Klenerman, David; Dobson, Christopher M; Mezzenga, Raffaele; Knowles, Tuomas P J; Aguzzi, Adriano A; Hornemann, Simone
Published in: Analytical Chemistry, Issue 89(22), 2017, Page(s) 12306-12313, ISSN 0003-2700
Publisher: American Chemical Society
DOI: 10.5167/uzh-141180

Prions, prionoids and protein misfolding disorders

Author(s): Claudia Scheckel, Adriano Aguzzi
Published in: Nature Reviews Genetics, Issue 19/7, 2018, Page(s) 405-418, ISSN 1471-0056
Publisher: Nature Publishing Group
DOI: 10.1038/s41576-018-0011-4

Intrinsic Toxicity of Antibodies to the Globular Domain of the Prion Protein

Author(s): Reimann, Regina R; Aguzzi, Adriano
Published in: Biological Psychiatry, Issue 12 April 2018, 2018, ISSN 0006-3223
Publisher: Elsevier BV
DOI: 10.5167/uzh-151650

A neuroprotective role for microglia in prion diseases

Author(s): Caihong Zhu, Uli S. Herrmann, Jeppe Falsig, Irina Abakumova, Mario Nuvolone, Petra Schwarz, Katrin Frauenknecht, Elisabeth J. Rushing, Adriano Aguzzi
Published in: The Journal of Experimental Medicine, Issue 213/6, 2016, Page(s) 1047-1059, ISSN 0022-1007
Publisher: Rockefeller University Press
DOI: 10.1084/jem.20151000

Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein

Author(s): Henning Leske, Simone Hornemann, Uli Simon Herrmann, Caihong Zhu, Paolo Dametto, Bei Li, Florent Laferriere, Magdalini Polymenidou, Pawel Pelczar, Regina Rose Reimann, Petra Schwarz, Elisabeth Jane Rushing, Kurt Wüthrich, Adriano Aguzzi
Published in: PLOS ONE, Issue 12/2, 2017, Page(s) e0170503, ISSN 1932-6203
Publisher: Public Library of Science
DOI: 10.1371/journal.pone.0170503

Cell Biology of Prions and Prionoids: A Status Report

Author(s): Adriano Aguzzi, Asvin K.K. Lakkaraju
Published in: Trends in Cell Biology, Issue 26/1, 2016, Page(s) 40-51, ISSN 0962-8924
Publisher: Elsevier BV
DOI: 10.1016/j.tcb.2015.08.007

Neurotoxic Antibodies against the Prion Protein Do Not Trigger Prion Replication

Author(s): Karl Frontzek, Manuela Pfammatter, Silvia Sorce, Assunta Senatore, Petra Schwarz, Rita Moos, Katrin Frauenknecht, Simone Hornemann, Adriano Aguzzi
Published in: PLOS ONE, Issue 11/9, 2016, Page(s) e0163601, ISSN 1932-6203
Publisher: Public Library of Science
DOI: 10.1371/journal.pone.0163601

The biological function of the cellular prion protein: an update

Author(s): Marie-Angela Wulf, Assunta Senatore, Adriano Aguzzi
Published in: BMC Biology, Issue 15/1, 2017, ISSN 1741-7007
Publisher: BioMed Central
DOI: 10.1186/s12915-017-0375-5

Strictly co-isogenic C57BL/6J- Prnp −/− mice: A rigorous resource for prion science

Author(s): Mario Nuvolone, Mario Hermann, Silvia Sorce, Giancarlo Russo, Cinzia Tiberi, Petra Schwarz, Eric Minikel, Despina Sanoudou, Pawel Pelczar, Adriano Aguzzi
Published in: The Journal of Experimental Medicine, Issue 213/3, 2016, Page(s) 313-327, ISSN 0022-1007
Publisher: Rockefeller University Press
DOI: 10.1084/jem.20151610

Differential Toxicity of Antibodies to the Prion Protein

Author(s): Regina R. Reimann, Tiziana Sonati, Simone Hornemann, Uli S. Herrmann, Michael Arand, Simon Hawke, Adriano Aguzzi
Published in: PLOS Pathogens, Issue 12/1, 2016, Page(s) e1005401, ISSN 1553-7374
Publisher: Public Library of Science
DOI: 10.1371/journal.ppat.1005401

Genome-Wide Identification of microRNAs Regulating the Human Prion Protein

Author(s): Pease, Daniel; Scheckel, Claudia; Schaper, Elke; Eckhardt, Valeria; Emmenegger, Marc; Xenarios, Ioannis; Aguzzi, Adriano
Published in: Pease, Daniel; Scheckel, Claudia; Schaper, Elke; Eckhardt, Valeria; Emmenegger, Marc; Xenarios, Ioannis; Aguzzi, Adriano (2019). Genome-Wide Identification of microRNAs Regulating the Human Prion Protein. Brain Pathology, 29(2):232-244., Issue 1, 2019, ISSN 1015-6305
Publisher: International Society of Neuropathology
DOI: 10.5167/uzh-158645

NG2 glia are required for maintaining microglia homeostatic state

Author(s): Yingjun Liu, Adriano Aguzzi
Published in: Glia, Issue 68/2, 2019, Page(s) 345-355, ISSN 0894-1491
Publisher: John Wiley & Sons Inc.
DOI: 10.1002/glia.23721

GPR56/ADGRG1 regulates development and maintenance of peripheral myelin

Author(s): Ackerman, Sarah D.; Luo, Rong; Poitelon, Yannick; Mogha, Amit; Harty, Breanne L.; D’Rozario, Mitchell; Sanchez, Nicholas E.; Lakkaraju, Asvin K.K.; Gamble, Paul; Li, Jun; Qu, Jun; MacEwan, Matthew R.; Ray, Wilson Zachary; Aguzzi, Adriano; Feltri, M. Laura; Piao, Xianhua; Monk, Kelly R.
Published in: Journal of Experimental Medicine, Issue 1, 2018, Page(s) 215(3):941-961., ISSN 0022-1007
Publisher: Rockefeller University Press
DOI: 10.5167/uzh-150267

Toxic Protein Spread in Neurodegeneration: Reality versus Fantasy

Author(s): Eleanna Kara, Jordan D. Marks, Adriano Aguzzi
Published in: Trends in Molecular Medicine, Issue 24/12, 2018, Page(s) 1007-1020, ISSN 1471-4914
Publisher: Elsevier BV
DOI: 10.1016/j.molmed.2018.09.004

Homozygous calreticulin mutations in patients with myelofibrosis lead to acquired myeloperoxidase deficiency

Author(s): Alexandre P. A. Theocharides, Pontus Lundberg, Asvin K. K. Lakkaraju, Veronika Lysenko, Renier Myburgh, Adriano Aguzzi, Radek C. Skoda, Markus G. Manz
Published in: Blood, Issue 127/25, 2016, Page(s) 3253-3259, ISSN 0006-4971
Publisher: American Society of Hematology
DOI: 10.1182/blood-2016-02-696310

Enhanced detection of prion infectivity from blood by preanalytical enrichment with peptoid-conjugated beads

Author(s): Hornemann, Simone; Schwarz, Petra; Rushing, Elisabeth J.; Connolly, Michael D.; Zuckermann, Ronald N.; Yam, Alice Y.; Aguzzi, Adriano
Published in: PLoS ONE, Vol 14, Iss 9, p e0216013 (2019), Issue 2, 2019, ISSN 1932-6203
Publisher: Public Library of Science
DOI: 10.5167/uzh-177400

Immunotherapy for neurodegeneration?

Author(s): Liu, Yingjun; Aguzzi, Adriano
Published in: Liu, Yingjun; Aguzzi, Adriano (2019). Immunotherapy for neurodegeneration? Science, 364(6436):130-131., Issue 1, 2019, ISSN 0036-8075
Publisher: American Association for the Advancement of Science
DOI: 10.1126/science.aaw0685

Ribosomal profiling during prion disease uncovers progressive translational derangement in glia but not in neurons

Author(s): Claudia Scheckel; Marigona Imeri; Petra Schwarz; Adriano Aguzzi
Published in: eLife, Issue 1, 2020, ISSN 2050-084X
Publisher: eLife Sciences Publications
DOI: 10.5167/uzh-190429

Autoantibodies against the prion protein in individuals with PRNP mutations

Author(s): Karl Frontzek, Manfredi Carta, Marco Losa, Mirka Epskamp, Georg Meisl, Alice Anane, Jean-Philippe Brandel, Ulrike Camenisch, Joaquín Castilla, Stéphane Haïk, Tuomas Knowles, Ewald Lindner, Andreas Lutterotti, Eric Vallabh Minikel, Ignazio Roiter, Jiri G. Safar, Raquel Sanchez-Valle, Dana Žáková, Simone Hornemann, Adriano Aguzzi
Published in: Neurology, 2020, Page(s) 10.1212/WNL.0000000000009183, ISSN 0028-3878
Publisher: Lippincott Williams & Wilkins Ltd.
DOI: 10.1212/wnl.0000000000009183

Genome-wide transcriptomics identifies an early preclinical signature of prion infection

Author(s): Silvia Sorce, Mario Nuvolone, Giancarlo Russo, Andra Chincisan, Daniel Heinzer, Merve Avar, Manuela Pfammatter, Petra Schwarz, Mirzet Delic, Micha Müller, Simone Hornemann, Despina Sanoudou, Claudia Scheckel, Adriano Aguzzi
Published in: PLOS Pathogens, Issue 16/6, 2020, Page(s) e1008653, ISSN 1553-7374
Publisher: Public Library of Science (PLoS)
DOI: 10.1371/journal.ppat.1008653

Anti-prothrombin autoantibodies enriched after infection with SARS-CoV-2 and influenced by strength of antibody response against SARS-CoV-2 proteins.

Author(s): Marc Emmenegger; Sreedhar S. Kumar; Vishalini Emmenegger; Tomas Malinauskas; Thomas Buettner; Laura Rose; Peter Schierack; Martin F. Sprinzl; Clemens Sommer; Karl J. Lackner; Adriano Aguzzi; Dirk Roggenbuck; Katrin Frauenknecht
Published in: PLoS Pathogens, Issue 2, 2021, ISSN 1553-7366
Publisher: Public Library of Science
DOI: 10.5167/uzh-211255