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Function and malfunction of the prion protein

Total Cost €


EC-Contrib. €






 PRION2020 project word cloud

Explore the words cloud of the PRION2020 project. It provides you a very rough idea of what is the project "PRION2020" about.

basis    prove    applicable    peripheral    sequencing    crispr    cellular    mutations    spongiform    dearth    screens    principles    translate    function    infected    er    lethality    clarifying    perform    assay    rising    molecular    perhaps    instalment    pathology    ordered    parkin    gen    vacuolation    unbiased    yield    genetic    uncover    familial    cell    prions    employing    variety    perspectives    lines    tools    coupled    receptor    genes    alzheimer    therapeutics    neuronal    certain    consisting    synthetic    parkinson    integrity    li    caused    operate    encephalopathies    son    transmissible    neurons    prpsc    alleys    protein    aggregation    diagnostics    sirna    quantitative    secretion    braries    disease    expressed    degeneration    biology    myelin    invariably    neurotoxicity    trigger    hence    spongiosis    genome    undoubtedly    physiology    interacts    tse    genetics    understand    orphan    pathogenetic    stressors    modifiers    steadily    throughput    list    replication    suspect    erc    first    utilizes    death    driver    diseases    infections    toxicity    insights    prpc    prevalence    hampered    prion   

Project "PRION2020" data sheet

The following table provides information about the project.


Organization address
address: RAMISTRASSE 71
city: Zürich
postcode: 8006

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country Switzerland [CH]
 Total cost 2˙500˙000 €
 EC max contribution 2˙500˙000 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2014-ADG
 Funding Scheme ERC-ADG
 Starting year 2015
 Duration (year-month-day) from 2015-11-01   to  2020-10-31


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    UNIVERSITAT ZURICH CH (Zürich) coordinator 2˙500˙000.00


 Project objective

Transmissible spongiform encephalopathies (TSE) are caused by the ordered aggregation of PrPC into prions consisting of PrPSc. Similar pathogenetic principles operate in Alzheimer’s and Parkin-son’s disease, and a growing list of further diseases whose prevalence is steadily rising. Familial TSE are invariably associated with PrPC mutations, and the dearth of genetic modifiers has hampered our understanding of prion diseases. Therefore, the first objective of my proposal utilizes a cell-based high-throughput quantitative prion replication assay (developed during my previous ERC instalment) for genome-wide unbiased screens employing new genetics tools (CRISPR, siRNA li-braries, next-gen sequencing) to identify modifiers of prion uptake, replication, and secretion. The second objective aims at clarifying the basis of prion neurotoxicity and will be developed along two alleys: (a) we will uncover the molecular basis of spongiosis (the neuronal vacuolation characteristic of prion diseases), which we suspect to be a main driver of pathology, and (b) we will perform CRISPR-based synthetic lethality screens to identify genes that become essential to prion-infected cell lines (which do not experience prion toxicity) and may not be expressed by neurons. The third objective is to understand the function of PrPC in cellular physiology, and focuses on our evidence that (a) PrPC interacts with an orphan G-protein coupled receptor to maintain peripheral myelin integrity and (b) that PrPC may trigger cell death in response to ER stressors. While certain pathways of degeneration will undoubtedly be specific to prion infections, I expect that some targets will prove common to a variety of protein aggregation diseases including Alzheimer’s and Parkinson’s disease, and may perhaps translate into novel diagnostics and therapeutics. Hence the proposed project may not only open new perspectives in prion biology but also yield insights applicable to much more common diseases.


year authors and title journal last update
List of publications.
2017 Marie-Angela Wulf, Assunta Senatore, Adriano Aguzzi
The biological function of the cellular prion protein: an update
published pages: , ISSN: 1741-7007, DOI: 10.1186/s12915-017-0375-5
BMC Biology 15/1 2019-07-05
2017 Ballmer, Boris A.; Moos, Rita; Liberali, Prisca; Pelkmans, Lucas; Hornemann, Simone; Aguzzi, Adriano
Modifiers of prion protein biogenesis and recycling identified by a highly parallel endocytosis kinetics assay
published pages: 8356-8368., ISSN: 0021-9258, DOI: 10.5167/uzh-136555
Journal of Biological Chemistry 292(20) 2019-07-05
2016 Mario Nuvolone, Mario Hermann, Silvia Sorce, Giancarlo Russo, Cinzia Tiberi, Petra Schwarz, Eric Minikel, Despina Sanoudou, Pawel Pelczar, Adriano Aguzzi
Strictly co-isogenic C57BL/6J- Prnp −/− mice: A rigorous resource for prion science
published pages: 313-327, ISSN: 0022-1007, DOI: 10.1084/jem.20151610
The Journal of Experimental Medicine 213/3 2019-07-05
2016 Karl Frontzek, Manuela Pfammatter, Silvia Sorce, Assunta Senatore, Petra Schwarz, Rita Moos, Katrin Frauenknecht, Simone Hornemann, Adriano Aguzzi
Neurotoxic Antibodies against the Prion Protein Do Not Trigger Prion Replication
published pages: e0163601, ISSN: 1932-6203, DOI: 10.1371/journal.pone.0163601
PLOS ONE 11/9 2019-07-05
2018 Adriano Aguzzi, Asvin K.K. Lakkaraju, Karl Frontzek
Toward Therapy of Human Prion Diseases
published pages: 331-351, ISSN: 0362-1642, DOI: 10.1146/annurev-pharmtox-010617-052745
Annual Review of Pharmacology and Toxicology 58/1 2019-07-05
2018 Claudia Scheckel, Adriano Aguzzi
Prions, prionoids and protein misfolding disorders
published pages: 405-418, ISSN: 1471-0056, DOI: 10.1038/s41576-018-0011-4
Nature Reviews Genetics 19/7 2019-07-05
2016 Caihong Zhu, Uli S. Herrmann, Jeppe Falsig, Irina Abakumova, Mario Nuvolone, Petra Schwarz, Katrin Frauenknecht, Elisabeth J. Rushing, Adriano Aguzzi
A neuroprotective role for microglia in prion diseases
published pages: 1047-1059, ISSN: 0022-1007, DOI: 10.1084/jem.20151000
The Journal of Experimental Medicine 213/6 2019-07-05
2016 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
The prion protein is an agonistic ligand of the G protein-coupled receptor Adgrg6
published pages: , ISSN: 0028-0836, DOI: 10.5167/uzh-125485
Nature 4 2019-07-05
2017 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
Absolute Quantification of Amyloid Propagons by Digital Microfluidics
published pages: 12306-12313, ISSN: 0003-2700, DOI: 10.5167/uzh-141180
Analytical Chemistry 89(22) 2019-07-05
2017 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
Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity
published pages: e1006733, ISSN: 1553-7374, DOI: 10.1371/journal.ppat.1006733
PLOS Pathogens 13/11 2019-07-05
2017 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
Protease resistance of infectious prions is suppressed by removal of a single atom in the cellular prion protein
published pages: e0170503, ISSN: 1932-6203, DOI: 10.1371/journal.pone.0170503
PLOS ONE 12/2 2019-07-05
2016 Regina R. Reimann, Tiziana Sonati, Simone Hornemann, Uli S. Herrmann, Michael Arand, Simon Hawke, Adriano Aguzzi
Differential Toxicity of Antibodies to the Prion Protein
published pages: e1005401, ISSN: 1553-7374, DOI: 10.1371/journal.ppat.1005401
PLOS Pathogens 12/1 2019-07-05
2018 Reimann, Regina R; Aguzzi, Adriano
Intrinsic Toxicity of Antibodies to the Globular Domain of the Prion Protein
published pages: , ISSN: 0006-3223, DOI: 10.5167/uzh-151650
Biological Psychiatry 12 April 2018 2019-07-05
2016 Adriano Aguzzi, Asvin K.K. Lakkaraju
Cell Biology of Prions and Prionoids: A Status Report
published pages: 40-51, ISSN: 0962-8924, DOI: 10.1016/j.tcb.2015.08.007
Trends in Cell Biology 26/1 2019-07-05

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