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Please use the program below to view each day of the conference.

Please note that this is the provisional programme and may be subject to change.

Workshop 1 Lecture Hall 103

Workshop on Animal prion diseases: Emerging Prion Diseases Surveillance, Detection, Pathogenesis

The focus of this workshop will be ongoing studies and findings that enhance our understanding about the emergence and transmission of animal prion diseases. Special emphasis will be placed on short oral talks from early career, students and technicians.

09:00h Welcome
09:10h S. Benestad: Norwegian Veterinary Institute, Emerging CWD strains in Europe, overall view of current/emerging situation
09:35h G. Telling: A diverse spectrum of novel strains among Nordic cervids with chronic wasting disease
09:55h D. Walter: Strain Types of Chronic Wasting Disease and Efforts Towards a Virtual Tissue Repository
10:15h A. Huor: ARR/ARR genotype sheep show no resistance to ovine adapted c-BSE infection by the oral route
10:35h
Coffee Break
11:00h C. Mathiason: Colorado State University, Seeking biological relevance of CWD maternal infections and transmission from dam to offspring
11:25h F. Houston: Subclinical infection in sheep exposed to low doses of prions by blood transfusion
11:45h E. Vidal: Bona fide spontaneous and atypical scrapie faitfully reproduced through the expression of a polymorphic variant of ovine prion protein
12:25h Concluding remarks
Workshop 2 Lecture Hall 104

Structural biology of protein misfolding diseases

Recent advances in cryo electron microscopy and other techniques have given us detailed insights into the structures of amyloid fibrils that were either isolated from diseased brains or generated in vitro. The question, which of theses structures represent the biologically active form, can be difficult to answer.

In this workshop, we will discuss the latest technical approaches to analyze the structures of amyloid fibrils and other misfolded proteins. We welcome contributions describing structural analyses at low and high resolutions, as well as studies that investigate the question of biological activity.

Special emphasis will be given to short oral presentations by trainees (all levels) to encourage young structural biologists in their quest to analyze the structures of amyloid fibrils and other protein aggregates.

09:00h  Welcome Requena/Wille
09:05h A. Kraus: TBD Prion structure
09:35h M. Zweckstetter: Tau – a key target to treat Alzheimer’s disease
10:05h M. Rigoli: Computational Paradigms to Study Prion Folding & Misfolding
10:20h H. Eraña: Understanding the key features of the spontaneous formation of bona fide recombinant prions through a new method allowing their consistent generation within hours
10:35h Coffee break
10:55h V. Rathod: In vitro refolding of the 7kDa A117V GSS peptide
11:10h F. Wang: Faithful propagation of prion strain-specific conformation to recombinant protein
11:25h H. Rezai: Strain determinant minimal substructure revealed by dissociation of PrPSc assemblies
11:40h M. Rayner: Prion propagation is dependent upon key N-terminal amino acids within the prion protein
11:55h H. Wille: Infectious prions – are they all PIRIBS structures now?
12:25h Concluding remarks (Requena / Wille)
Workshop 3 Different venue only for workshop 3: Universitätsmedizin Göttingen (UMG), Robert Koch Str.40, 37075 Göttingen, Room 01.E1.257 – DIPS3

Neuropathology and clinicopathological correlation of human prion diseases and related dementias.

Several dementias are characterized by aggregation of abnormally folded conformers of host encoded proteins. In prion diseases and related dementias, seeded aggregation can lead to the spread of protein aggregates throughout the brain.
In this workshop, we will focus on neuropathology, selective cellular and regional vulnerability, and clinicopathological correlation not only in Creutzfeldt-Jakob disease but also in Parkinson’s and Alzheimer’s disease. Besides lectures by prominent experts in the field such as Ellen Gelpi, Piero Parchi and Markus Glatzel there will be room for case discussions and hands-on neuropathology training in this exciting field of science.

14:00h M. Glatzel: Intruduccion into the concept of this workshop
14:05h P. Parchi: Histopathological and molecular variability in Creutzfeldt-Jakob disease: the effect of prion strain, host genotype, and disease etiology
14:30h E. Gelpi: alpha-synuclein neuroanatomical distribution in Parkinson’s disease
14:55h M. Glatzel: beta-amyloid and prion protein interactions and what this means for Creutzfeldt-Jakob and Alzheimers disease
15:15h
Coffee Break
15:30h Case discussion and hands-on neuropathology training. Cases will be presented by Ellen Gelpi, markus Glatzel and Piero Parchi and these cases will cover the topics of COVID-19 and sCJD. You have the chance to present your own interesting or puzzeling cases (in case you plan to do this, could you please inform us in advance by E-Mail)
16:45h M. Glatzel: Summary and feedback
Workshop 4 Lecture Hall 104

Bio-marker/Human diseases

The focus of this workshop will be ongoing studies on developments of seeding aggregation assays in neurodegenerative diseases. Special attention will be paid to clinical studies and improvement for early detection in various biological fluids and tissues in humans.

14:00h A. Green: SAA for alpha-synuclein
14:20h G. Zanusso: Human Prion diseases diagnosis by RT-QuIC
14:40h I. Zerr/M. Schmitz: Seeding conversion variability of misfolded tau conformers in classical and rapidly progressive Alzheimer’s disease
15:00h R. Sánchez-Valle: Quantitative 14-3-3 protein and prion RT-QuIC concordance analysis of patients with suspected prion diseases in Spain
15:15h N. Omer: Cerebrospinal fluid (CSF) and Plasma Biomarkers in patients with genetic Creutzfeldt-Jakob disease (gCJD) and healthy relatives, carriers of the E200K mutation: Results from an ongoing longitudinal study.
15:30h N. Younas: Early preclinical proteomic signatures of prion infection
15:45h Coffee break
16:15h C. N. Kraft: Nasal swab detection of prion shedding in CWD-infected white-tailed
16:30h C. M. Thomas: Comparison of in vitro tests (PMCA and RT-QuIC) and bioassay for longitudinal prion detection in preclinical blood samples from BSE infected sheep
16:45h D. F. Browne: Hypochlorous acid solutions reduce disease-associated tau seeding activity
17:00h S. Galušková: Evaluation of the seeding activity of alpha-synuclein in brain and cerebrospinal fluid tissue samples
17:15h L. Concha: Semi-quantitative αS-SAA detects no difference in αSyn seeds in CSF from prodromal to phenocon- version in longitudinal samples
17:30h M. Rossi: Towards an improved ‘quantitative’ α-synuclein Real-Time Quaking-Induced Conversion assay to assess Lewy body pathology in vivo
17:45h O. Bannach: Combination of seeded aggregation and sFIDA for diagnostics of neurodegenerative diseases
Session 1  Hall 011 Protein Structure– Function, Conversion, Dysfunction Chairs: D. Riesner/N. Lopez-Lorenzo
09:00h B. Caughey: Prion structures Keynote
09:45h E. Artikis: Understanding the Conformational Dynamics of Infectious Prion Fibrils 1.1
10:00h S. Manka: A pipeline for atomic structure determination of infectious ex vivo prion fibrils by cryo-EM 1.2
10:15h Y. Chernoff: Yeast models for studying aggregation of proteins, involved in Alzheimer’s disease 1.3
10:30h Coffee break
Session 2 Hall 011 Pathogenic mechanisms in tauopathies Chairs: M. Jucker/S. Krasemann
11.00h K. Duff: Mechanisms for the spread of tauopathies in AD and FTD Keynote
11:45h R. Chiesa: Inoculation of human traumatic brain injury tissue homogenates induces cognitive deficits and widespread tau pathology in wild-type mice 2.1
12:00h A. Kraus: Tau seeds precede earliest Alzheimer’s changes and are prevalent in synucleinopathies andother neurodegenerative diseases 2.2
12:15h Lunch
Session 3 Hall 011 Pathogenic mechanisms in synucleinopathies Chairs: T. Outeiro/G. Zanusso
14:00h A. L. Woerman: Alpha-synuclein prions in multiple system atrophy Keynote
14:45h J. Ayers: Different Alpha-Synuclein Prion Strains Cause Dementia with Lewy Bodies and Multiple System Atrophy 3.1
15:00h Ch. Orru: Performance of alpha-synuclein RT-QUIC in relation to neuropathological staging of Lewy body disease 3.2
15:15h L. Blömeke: Quantitative Detection of α-Synuclein and Tau Oligomers and other Aggregates by Digital Single Particle Counting 3.3
15.30h Coffee break
Session 4 Hall 011 Novel models for studying prion Chairs: M. Glatzel/I. Zerr
16:00h  O. Andréoletti: E200K CJD: a „model“ for studying sCJD? Keynote
16.45h E. Comoy: Non-human primates: a renewed gold standard for prion(-like) diseases? 4.1
17:00h A. Nihat: A dividing cell model for stable propagation and curing of bona fide human sporadic Creutzfeldt-Jakob Disease prions 4.2
17:15h D. Bougard: Correlation between bioassay and PMCA for human prion decontamination studies 4.3
Talk Hall 011 Special Lecture
17:30h P. Liberski: Kuru – where all the prion research began
Session 5 Hall 011 Structural biology of prions Chairs: H. Wille/H. Altmeppen
09:00h J. Requena/R. Riek: Structure-Activity Relationship of Amyloids Keynote
09.45h G. Jackson: Synthetic prions with high specific infectivity generated from recombinant PrP 5.1
10:00h V. Beringue: Efficient propagation and strain diversity of prions from pure synthetic origin 5.2
10:15h J. Bieschke: Direct Observation of Prion Protein Fibril Elongation Kinetics Reveals Competing Fibril Populations with Distinct Strain-like Structural and Dynamic Properties
5.3
10:30h Coffee break
Session 6 Hall 011 Novel molecular mechanisms in prion diseases Chairs: R. Chiesa/ I.Vorberg
11:00h A. Aguzzi: Genome-wide perturbations in prion science  Keynote
11:45h S. Mead: Genome wide association study of clinical duration and age at onset of sporadic CJD 6.1
12:00h J. Tatzelt: Liquid-liquid phase separation of the prion protein promotes the formation of neurotoxic aggregates; a critical role of the N-terminal domain 6.2
12:15h JM. Ribes: Prion protein converts at two distinct cellular sites and precedes fibril formation 6.3
12:30h Lunch
Session 7 Hall 011 Pathogenic mechanisms in ß Amyloidosis Chairs: C. Lasmezas/T. Sklaviadis
14:00h L. Walker: The prion paradigm, Abeta, and Alzheimer’s disease  Keynote
14:45h R. Gomez-Gutierrez: Structure-defined Aβ polymorphs promote different pathological changes in susceptible mice. 7.1
15:00h C. Korth: Title: Abeta dimers are antiprions that interfere with seeded nucleation in vitro and in vivo 7.2
15:15h M. Shafiq: Extracellular vesicles in the pathophysiology of Alzheimer’s disease: understanding the role of the prion protein 7.3
15:30h  Coffee break
Session 8 Hall 011 Function, dysfunction and conversion: from strains to transmission Chairs: J. Torres/A. Ruiz-Riquelme
16:00h J. Collinge: Understanding prion structure, strains and neurotoxicity  Keynote
16:45h M. Arifin: Heterozygosity at cervid Prnp codon 138 progressively blocks prion conversion in vitro and partly confines prion propagation to the periphery in knock-in mice 8.1
17:00h E. Cassmann: The chronic wasting disease agent from white-tailed deer is infectious to humanized mice after passage through raccoons 8.2
17:15h H. Schaetzl: Transmission of prion infectivity from CWD-infected macaque tissues to rodent models demonstrates the zoonotic potential of chronic wasting disease. 8.3
Talk Hall 011 Special Lecture by Industry
17:30h P. Perin (Quanterix): Simoa Technology for Ultrasensitive Biomarker Detection
Poster Party
Session 9 Hall 011 The interplay between Abeta and prion Chairs: J. Requena/D. Bougard
09.00h SM. Strittmacher: Amyloid-ß Interaction with Cellular Prion Protein in Alzheimer’s Disease  Keynote
09:45h G. Merz: Cryo-EM reveals small-molecule binding to the paired helical fillament conformation of tau prions from Alzheimer’s disease 9.1
10:00h S. Liu: De-repression of endogenous retroviruses promotes prion-like spreading of proteopathic seeds 9.2
10:15h  Coffee break
Session 10A Hall 011 CJD International Support Alliance Chairs: B. Appleby/P. Hermann
11:00h S. Solvyns (CJDISA) Keynote
11:30h J. & R. Backer: CJK-Initiative, Germany 10A.1
11:45h J. Castilla: Spanish Foundation for prion diseases 10A.2
Session 10B Hall 011 Genetic Prion Diseases Chairs: B. Appleby/P. Hermann
12:00h N. Majbour: Defining the onset of prion infection and neurodegeneration in healthy individuals at risk of prion disease 10B.3
12:15h A. Anane: Biobank of genetic CJD at Israel 10B.4
12:30h Lunch
Session 11A Hall 011 Therapeutic perspectives in prion diseases Chairs: P. Cras/J. Castilla
13:45h H. Zhao: ASO-mediated PrP suppression as disease modifying therapy for prion disease 11A.1
14:00h V. Bonaldo: Folding intermediates of the cellular PrP across disease and therapy 11A.2
14:15h S. Krasemann: mGluR5 inhibition delays cognitive decline and incubation time in a mouse model of prion disease 11A.3
14:30h B. Zeitler: Engineered zinc finger protein transcription factors potently reduce brain PrP expression and extend survival in prion-infected mice 11A.4
14:45h K. Xanthopoulos: Evaluation of the therapeutic action of poly(propylen Imine) glycodendrimers in prion disease mouse model 11A.5
15:00h R. Mercer: Two pronged pharmacological interventions for prion disease targeting propagation and toxicity 11A.6
15:15h M. Fleming: Optimizing prion vaccination in a transgenic mouse model of Gerstmann-Sträussler-Scheinker disease 11A.7
15:30h Coffee break
Session 11B  Hall 010 Animal Diseases Chairs: H. Schaetzl/C. Mathiason
13:45 S. Canoyra:Conformational shift at the evolutionary mechanism for classical BSE emergence from atypical scrapie 11B.1
14:00h G. Telling: Divergent strain profiles of European and North American CWD 11B.2
14:15h R. Morales: Nasal bot: an emerging vector for natural chronic wasting disease transmission 11B.3
14:30h N. Denkers: Shedding of Chronic Wasting Disease Prions in Multiple Excreta Throughout Disease Course in White-tailed Deer   11B.4
14:45h R. Bujdoso: A new bioassay for the sensitive detection of blood-borne CWD prions  11B.5
15:00h J. Greenlee: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation 11B.6
15:30h Coffee break
Session 12 Hall 011 hot topics/breaking news/controversies Chairs: Chr. Orru/E. Gelpi
16:00h S. Hannaoul: Transmission of Cervid Prions to Humanized Mice Demonstrates the Zoonotic Potencial of CWD 12.1
16:15h W. Q. Zou: Generation of human chronic wasting diseases in transgenic mice 12.2
16:30h A. Castle: Beta-endoproteolysis of the cellular prion protein by dipeptidyl peptidase-4 and fibroblast activation protein 12.3
16:45h G. Jansen: Neuropathology of 8 patients of the New Brunswick cluster of Neurological Syndrome of Unknown Cause; human Chronic Wasting Disease or blue-green algae? 12.4
17:00h C. Lasmezas: A novel neuroprotective approach for protein misfolding neurodegenerative diseases 12.5
17:15h Poster Award
17:30h Prion 2023/End of the meeting