ProgramMay 10 (Tue.)
Animal Prion Diseases Workshop“Updated Diagnosis and Epidemiology of Animal Prion Diseases for Food Safety and Security”(Sponsored by the OECD Co-operative Research Programme)

9:00-9:05

Opening Remarks

9:05-9:15

OECD programme
Rafael Blasco
INIA-Ministerio de Ciencia e Innovacion

9:15-10:50

Workshop 01-04

Chairs: Motohiro Horiuchi (Hokkaido University)
		Corinne Lasmezas (The Scripps Research Institute)
WS-01

Prion diseases in animals and zoonotic potential
Juan Maria Torres
Centro de Investigacion en Sanidad Animal (CISA-INIA), Valdeolmos, Madrid, Spain

WS-02

Scrapie in swine: A diagnostic challenge
Justin J Greenlee
National Animal Disease Center, US Dept. of Agricultrue, Agricultural Research Service, United States

WS-03

Epidemiology of Chronic wasting disease in Korea
Hyun-Joo Sohn
Foreign Animal Disease Division (FADD), Animal and Plant Quarantine Agency (QIA), Korea

WS-04

Ultra-sensitive detection of PrPSc of classical and atypical BSEs
Yuichi Murayama
Influenza/Prion Disease Research Center, National Institute of Animal Health, Japan

10:50-12:45

Workshop 05-08

Chairs: Yoshifumi Iwamaru (National Institute of Animal Health)		Jean Manson (The Roslin Institute, University of Edinburgh)
WS-05

RT-QuIC Assays in Humans and Animals
Steven J Collins
The University of Melbourne, Australia

WS-06

Pathology of TSEs
John Spiropoulos
Animal and Plant Health Agency, Pathology Department, United Kingdom

WS-07

Diagnosis of Atypical BSE and Isolation of the Agent
Sandor Dudas
Canadian National and OIE BSE Reference Laboratory, Canadian Food Inspection Agency, National Center for Animal
Disease, Lethbridge, Canada- 20 -

WS-08Intra- and interspecies transmission of atypical BSE - What can we learn from it?
Anne Balkema-BuschmannFriedrich-Loeffler-Institut, Institute for Novel and Emerging Infectious Diseases, Germany

Luncheon Seminar 1"Prion treatment by MC Water and other chemicals"*Co-sponsored by Santa Mineral Co., Ltd.
		
12:45-13:30

Chair: Koriki Jojima (Former Minister of Finance)
Speakers: Takashi Onodera (Professor of University of Tokyo)
		Kouichi Furusaki (General incorporated association Mineral Activation Technical Research Center)

13:30-15:00

Workshop 09-11

Chairs: Hiroyuki Okada (National Institute of Animal Health)		John Spiropoulos (Animal and Plant Health Agency)
WS-09

Pathogenesis and transmission of classical and atypical BSEs in cattle
Cristina Casalone
Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Italy

WS-10

Neuronal death and prion diseases
Corinne Lasmezas
Department of Immunology and Microbial Science, Department of Neuroscience, The Scripps Research Institute,
United States

WS-11

Evaluating the species barriers of TSEs with transgenic mouse models
Jean C Manson
The Roslin Institute, University of Edinburgh, United Kingdom

15:00-15:30

Closing Discussion

Chairs: Toshiro Kawashima (Ministry of Agriculture, Forestry and Fisheries, Japan)
		Takashi Yokoyama (National Institute of Animal Health, NARO)

15:45-16:15

Opening Lecture

Chair: Motomasa Tanaka (RIKEN Brain Science Institute, Lab for Protein Conformation Diseases)
OL-01

Implications of the folded in-register parallel beta-sheet structure of infectious prionamyloids
Reed B. Wickner
National Institutes of Health; National Institutes of Diabetes and Digestive and Kidney Diseases; Laboratory ofBiochemistry and Genetics, United States

16:15-16:45

Opening Lecture

Chair: Naomi Hachiya (Tokyo Medical University)
OL-02

PrPC function and prion toxicity
Adriano Aguzzi
Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse, Zürich, Switzerland- 21 -

May 11 (Wed.)
9:00-11:00

Invited Lectures "Conversion and Propagation"

Chairs: Noriyuki Nishida (Molecular Microbiology and Immunology, Nagasaki University)
Glenn Telling (Colorado University)
IL-01

Molecular Determinants of Prions Infectivity
Giuseppe Legname
Laboratory of Prion Biology, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA),
Trieste, Italy

IL-02

Potential role of the environment on prion transmission: Plants, environmental
surfaces and earthworms as carriers of infectious prions
Claudio Soto
Mitchell Center for Alzheimer's Diseases and Related Brain Disorders, Department of Neurology, University of Texas
Medical School at Houston, United States

IL-03

How does recombinant prion protein become infectious?
Progress in understanding the molecular basis of prion infectivity
Jiyan Ma
Van Andel Institute, United States

IL-04

Sorting of prion protein and PrPSc accumulation
Keiji Uchiyama
Institute for Enzyme Research, Tokushima University, Japan

11:15-12:00
O-01

Oral Session
Folding and misfolding pathways of prion protein
Ryo P. Honda
Department of Molecular Pathobiochemistry, Gifu University Graduate School of Medicine, Japan

O-02

HET-2s, an engineered, four-rung beta-solenoid protein as a model for the structure of
PrPSc
Holger Wille
Centre for Prions & Protein Folding Diseases, Canada

O-03

Prion protein deficiency causes diverse proteome shifts in cell models that escapedetection in brain tissue
Mohadeseh MehrabianTanz Centre for Research in Neurodegenerative Diseases; Laboratory Medicine & Pathobiology, University of Toronto,
Toronto, ON, Canada

Luncheon Seminar 2"From prion diseases to Alzheimer’s disease"*Co-sponsored by Daiichi Sankyo Co., Ltd.
		
12:10-13:00

Chair: Hidehiro Mizusawa (President / National Center of Neurology and Psychiatry)
Speaker: Masahito Yamada (Professor and Chairman / Department of Neurology & Neurobiology ofAging, Kanazawa University Graduate School of Medical Sciences)- 22 -

13:00-14:30

Invited Lectures "Pathogenesis I"

Chairs: Byron Caughey (LPVD, Rocky Mountain Labs, NIAID, NIH)
Shigeo Murayama (Tokyo Metropolitan Institute of Gerontology)
IL-05

Prion structures, PIRIBS architectures, and real-time quaking-induced conversion (RTQuIC)
Byron Caughey
LPVD, Rocky Mountain Labs, NIAID, NIH, United States

IL-06

Neuropathology of prion disease
James W Ironside
National CJD Research & Surveillance Unit, University of Edinburgh, UK

IL-07

Iatrogenic transmission of Creutzfeldt-Jakob disease
Atsushi Kobayashi
Laboratory of Comparative Pathology, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo, Japan

14:45-16:15

Invited Lectures "Expansion of prion concept"

Chairs: Hidehiro Mizusawa (NCNP)
Joaquin Castilla (Proteomic, CICbioGUNE, Spain)
IL-08

Revisiting supersaturation as a factor determining amyloid fibrillation
Yuji Goto
Institute for Protein Research, Osaka University, Japan

IL-09

Mechanisms of prion protein (mis) folding and aggregation explored by ultrafast
kinetics
Heinrich Roder
Fox Chase Cancer Center, United States

IL-10

Tau / TDP-43 prions
Masato Hasegawa
Department of Dementia and Higher Brain Function, Tokyo Metropolitan Institute of Medical Science,
Setagaya-ku, Tokyo, Japan

16:15-17:00
O-04

Oral Session
Protein Folding Activity of the Ribosome: Key Player in Yeast Prion Propagation
Cecile P Voisset
Inserm UMR 1078, University of Medecine of Brest, Etablissement Français du Sang (EFS) Bretagne, France; CHRU
Brest, Morvan Hospital, Molecular Genetic Laboratory, Brest, France

O-05

A local conformation of natively disordered yeast prion monomer determinesinterspecies prion transmissibility
Toshinobu Shida
Department of Biological Information, Graduate School of Bioscience and Biotechnology, Tokyo Institute of Technology,
Yokohama, Japan; Laboratory for Protein Conformation Diseases, RIKEN Brain Science Institute, Wako, Japan

O-06

Prion nucleation and propagation by amyloid beta in the yeast model
Yury O. Chernoff
School of Biology, Georgia Institute of Technology, Atlanta, GA, USA; St. Petersburg State University, Russia- 23 -

May 12 (Thu.)
9:00-10:30

Invited Lectures "Pathogenesis II"

Chairs: Shirou Mohri (Department of Neurological Science, Tohoku University Graduate School of
Medicine)

Jean Manson (Neurobiology, The Roslin Institute, University of Edinburgh)
IL-11

Desigh of novel anti-prion compounds
Steven H. Olson
UCSF

IL-12

Activation state of glial cells in prion diseases
Motohiro Horiuchi
Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University, Japan

IL-13

Transmission of prions to non human-primates: Implications for human populations
Jean-Philippe Deslys
CEA, Institute of Emerging Diseases and Innovative Therapies (iMETI), Division of Prions and Related Diseases (SEPIA),
Fontenay-aux-Roses, France

10:45-12:00
O-07

Oral Session
Oral prion pathogenesis is reduced in the absence of CXCR5-expressing mononuclear
phagocytes
Neil A. Mabbott
The Roslin Institute & R(D)SVS, University of Edinburgh, UK

O-08

Tau pathology in Creutzfeldt-Jakob disease: Novel insights
Gabor G. Kovacs
Medical University of Vienna, Institute of Neurology, Austria

O-09

Creutzfeldt-Jakob disease prion propagation in human iPS cells-derived astrocytes
Zuzana Krejciova
Institute for Neurodegenerative Diseases, University of California, San Francisco, United States; National CJD Research& Surveillance Unit, University of Edinburgh, United Kingdom

O-10

Role of Tunneling Nanotubes (TNTs) in intercellular spreading of prions and otherprotein
Chiara Zurzolo
Pasteur Institute, Department of Cell Biology and Infection, France

O-11

Structural role of the middle region in the prion protein in conformational conversion
to the infectious form
Witold K Surewicz
Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA

Luncheon Seminar 3"Clinicopathological Feature of Genetic Prion Diseases in Japan"*Co-sponsored by Novartis Pharma K.K.
		
12:10-13:00

Chair: Yoshio Tsuboi (Professor of Department of Neurology, Fukuoka University)
Speaker: Nobuo Sanjo (Associate Professor of Department of Neurology and Neurological Science, Tokyo
Medical and Dental University)- 24 -

13:00-14:30

Invited Lectures "Human Prion Diseases: Diagnosis"

Chairs: Michael D. Geschwind (Neurology, University of California, San Francisco)
Ichirou Takumi (Nippon Medical School)
IL-14

Variant CJD
Robert G Will
National CJD Research and Surveillance Unit, United Kingdom

IL-15

Genetic prion diseases
Inga Zerr
National CJD Surveillance Center, Dept. of Neurology, Georg August University Göttingen, Germany

IL-16

Biomarkers for prion disease
Piero Parchi
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Italy; IRCCS, Istituto delle Scienze
Neurologiche di Bologna, Bologna, Italy

14:45-15:30
O-12

Oral Session
PrPSc in the skin of CJD patients
Wenquan Zou
Case Western Reserve University, United States

O-13

Autopsy validation of second generation RT QuIC for diagnosis and differentiationof human prion diseases: Results from the US National Prion Disease Pathology
Surveillance Center
Jiri G. Safar
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, United States

O-14

Highly sensitive and specific detection of prions in blood of vCJD patients by PMCA
Luis M Concha-Marambio
University of Texas Health Science Center, United States; Universidad de Los Andes, Facultad de Medicina, Chile

15:30-16:30

Invited Lectures "Animal Prion Diseases"

Chairs: Motohiro Horiuchi (Graduate School of Veterinary Medicine, Hokkaido University)
Candace Mathiason (Microbiology, Immunology and Pathology, Colorado State University)
IL-17

Emergence of a novel bovine spongiform encephalopathy (BSE) prion from an atypicalH-type BSE: An artificial laboratory strain or possible risk?
Takashi Yokoyama
National Insitute of Animal Health, NARO, Japan

IL-18

Rapid analysis of prion seeding activity in clinically accessible biological samples using
magnetic particle extraction and realtime conversion
Edward A Hoover
Microbiology, Immunology, Pathology, Colorado State University, United States

16:30-17:00
O-15

Oral Session
Zoonotic Potential of CWD Prions: An Update
Qingzhong Kong
Department of Pathology, Case Western Reserve University, Cleveland, Ohio, USA

O-16

Puzzling out the BSE-human transmission barrier
Natalia Fernandez-Borges
CISA-INIA, Valdeolmos, Madrid, Spain- 25 -

May 13 (Fri.)
9:00-11:00

Invited Lectures-APPS 2016 "Human Prion Diseases: Surveillance"

Chairs: Steve John Collins (Medicine, University of Melbourne, Australia)
Yoshikazu Nakamura (Department of Public Health, Jichi Medical University)
IL-19

Epidemiological and clinical features of human prion diseases in Japan: Prospective 17year surveillance
Masahito Yamada
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences,
Japan

IL-20

Chinese surveillance program for prion diseases
Xiao-Ping Dong
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control andPrevention, Chinese Center for Disease Control and Prevention, Beijing, China

IL-21

Surveillance of prion diseases in Taiwan
Shun-Sheng Chen
Department of Neurology, Kaohsiung Chang Gung Memorial Hospital, Taiwan

IL-22

Real-time quaking-induced conversion analysis for the diagnosis of sporadic
Creutzfeldt-Jakob disease in Korea
Yong-Sun Kim
Ilsong Institute of Life Science, Hallym University, Department of Neurodegenerative Diseases, Korea CJD Diagnostic
Center, Korea

11:15-12:05
O-17

Oral Session "APPS Cutting-edge young researcher"Acceleration of Abeta brain amyloidosis by peripheral administration of diseaseassociated aggregates
Rodrigo Morales
The University of Texas Health Science Center at Houston, United States

O-18

Amyloid beta pathology in iatrogenic Creutzfeldt-Jakob disease: A multi-center study
Ignazio Cali
Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, USA

O-19

Prion acute synaptotoxicity at the CA1 region of the stratum radiatum
Simote T Foliaki
Department of Medicine, The University of Melbourne, Australia

O-20

Neuron and glial cell type-specific detection of PrPSc in prion-infected mouse brain byflow cytometry
Takeshi Yamasaki
Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University, Japan

Luncheon Seminar 4 "α-Synuclein prions"*Co-sponsored by GlaxoSmithKline K.K.
		

12:10-13:00

Chair: TBD
Speaker: Masato Hasegawa (Department Head, Department of Dementia and Higher Brain Function
Tokyo Metropolitan Institute of Medical Science)- 26 -

13:00-15:00

Invited Lectures "Human Prion Diseases: Treatment"

Chairs: Neil Roy Cashman (Medicine (Neurology), University of British Columbia)
Yoshio Tsuboi (Fukuoka University)
IL-23

Therapeutic approaches to prion infection and disease
John Collinge
MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology and National Prion Clinic,
National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom

IL-24

Lessons from recent outcomes of clinical trials and therapeutic studies
Katsumi Doh-ura
Department of Neurochemistry, Tohoku University Graduate School of Medicine, Japan

IL-25

Prevention Trial in Fatal Familial Insomnia
Fabrizio Tagliavini
IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy

IL-26

Logical design of a therapeutic agent for prion diseases
Kazuo Kuwata
United Graduate School of Drug Discovery and Medical Information Sciences, Gifu University, Japan

15:15-16:00
O-21

Oral Session
Towards prophylactic treatments for carriers of pathogenic PrP mutations
Ruth Gabizon
Department of Neurology, Hadassah University Hospital, Israel; Granalix

O-22

Eradication of PrPSc by poly-L-arginine in cells infected with prions
Muhammad Waqas
Hanyang University, Korea

O-23

Pre-implantation exclusion of embryos at risk for prion diseases
Vardiella Meiner
Hadassah Hebrew University Hospital, Israel- 27 -

Poster Session
May 11 (Wed.)-May 12 (Thu.)
P-001

17:15-19:15

Employing Dynamic Mass Redistribution to Identify Pharmacological Chaperones for
The Cellular Prion Protein
Saioa R. Elezgarai
Department of Molecular Biochemistry and Pharmacology, IRCCS-Istituto di Ricerche Farmacologiche Mario Negri,
Spain; Dulbecco Telethon Institute, Centre for Integrative Biology (CIBIO), University of Trento, Trento, Italy

P-002

Using small molecule reagents to help distinguish among prion structural models
Christopher J. Silva
United States Department of Agriculture, ARS, WRRC, United States

P-003

PrP, in conjunction with CD21/35, bolsters B lymphocyte antibody responses, yet
infectious prions utilize this system for lymphoid replication
Sarah J Kane
Colorado State University, United States

P-004

Structural characterisation of ex vivo mammalian prions isolated from multiple strains
Cassandra I.J Terry
MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, United Kingdom

P-005

Lack of stress protection by the cellular prion protein: An alternative role in regulating
growth factor signalling
Andrew R Castle
Roslin Institute, University of Edinburgh, Edinburgh, UK

P-006

Structural and folding studies of the protective V127 variant of human prion protein
Laszlo LP Hosszu
MRC Prion Unit, United Kingdom

P-007

Non-equivalent binding sites for Abeta1-40 on PrP determine the oligomerisation
pathway
Katarina Grznarova
Brain and Spine Institute Paris, France; INRA/ National Institute of Agronomic Research, Molecular Virology and
Immunology (VIM), Protein Macro-assembly and prion diseases (MAP2), Domaine de Vilvert, Jouy-en-Josas, France;
CEA/French Alternative Energies and Atomic Energy Commission, Institute of Emerging Diseases, Innovative Therapies(SEPIA), Fontenay-aux-Roses, France

P-008

Proteolytic shedding of PrPC: Giving a little to gain a lot?
Hermann C Altmeppen
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

P-009

Calibration of ultrasonic power and conformational analysis of MoPrP amyloid fibrils
Kei-ichi Yamaguchi
United Graduate School of Drug Discovery and Medical Information Sciences, Gifu University, Japan; Center for
Emerging Infectious Diseases, Gifu University

P-010

Polymorphism Analysis of Prion Protein Gene in Eleven Pakistani Goat Breeds
Mohammad Farooque Hassan
China Agricultural University, Beijing, China; SBBUVAS, Sakrand Sindh Pakistan

P-011

Matrix metalloprotease processing of the prion protein
Victoria Lewis
Department of Medicine, RMH, The University of Melbourne, Australia- 28 -

P-012A ZIP6-ZIP10 heteromer interacts with NCAM1, controlling its phosphorylation andintegration into focal adhesion complexes during epithelial-to-mesenchymal transition
Dylan BrethourTanz Centre for Research in Neurodegenerative Diseases; Department of Laboratory Medicine & Pathobiology,
University of Toronto, Toronto, ON, Canada

P-013

Dimer-sized PrPSc formation detected by western blotting
Kenta Teruya
Department of Neurochemistry Tohoku University Graduate School of Medicine, Japan

P-014

The Prion Protein and genotoxic stress
Malin R. Reiten
Norwegian University of Life Sciences, Norway

P-015

Effects of cell growth suppression treatments on PrPSc accumulation in prion-infected
cells; Paradoxical phenomena observed in butyric acid treatment
Takako Hiyoshi
Department of Neurochemistry, Tohoku University Graduate School of Medicine, Japan

EP-001

A novel system for massively parallel, quantitative analysis of PrP interactions andbinding interfaces
Stefanie M. Berges
University of Delaware, United States

EP-002The influence of Prnpb polymorphisms and the conserved 4-threonine stretch of
Alpha-helix 2 on prion protein conversion
Romany Abskharon
Van Andel Research Institute, United States; National Institute of Oceanography and Fisheries (NIOF), Cairo, Egypt

EP-003

Solubilization of the aggregated-prion protein with the robust protein-unfolding
activity of an oligomeric form of YDL178wp/ Unfoldin
Naomi Hachiya
Tokyo Medical University, Pathophysiology, Japan

P-016

Manipulation of Autophagic machinery Controls Exosomal Release of Prions andLateral Prion Infection
Basant A Abdulrahman
Department of Camparative Biology and Experimental Medicine, Canada

P-017

Semisynthesis of lipidated prion protein variants
Stefanie Hackl
Institute of Biological Chemistry, Department of Chemistry, University of Vienna, Waehringer Str.,Vienna, Austria

P-018

In vitro seeding of amyloid plaques
Kirsty A Ireland
The Roslin Institute, United Kingdom

P-019

Discovery of anti-prion agents using a PyMOL plugin-based logical drug design
platform NAGARA
Biao Ma
United Graduate School of Drug Discovery and Medical Information Sciences, Gifu University, Gifu, Japan

P-020

Zinnia elegans combined PrPBSE complex increases the survival time of VM mice
Hyo Jin Kim
Foreign Animal Disease Division, Animal and Plant Quarantine Agency, Korea

P-021

Gene expression profiling analysis of Rubus coreanus Miquel-cured prion-infected
MDBK cell line
Hyo Jin Kim
Foreign Animal Disease Division, Animal and Plant Quarantine Agency, Korea- 29 -

P-022The effects of PrPC glycosylation and cofactor molecules on species-specific prion
strain susceptibility in the bank vole
Cassandra M Burke
Department of Biochemistry, Geisei School of Medicine at Dartmouth, Hanover, New Hampshire, United States

P-023

Autophagy is needed in opposing roles in the life cycle of prions and also impacts
exosomal release of prions
Hermann M Schatzl
University of Calgary, Comp. Biol. & Exp. Med., Canada

P-024

Polymorphism of PrP amyloid-like fibrils can be defined by the concentration of seeds
Vytautas Smirnovas
Vilnius University Institute of Biotechnology, Dept. Biothermodynamics and Drug Design, Lithuania

P-025

Restricted propagation of sheep scrapie in hamsters
Ronald A Shikiya
Creighton University, Medical Microbiology and Immunology, United States

P-026

Comparison of the in vitro seeding activity of UK iatrogenic and sporadic CreutzfeldtJakob disease subtypes by real time quaking induced conversion
Alexander H Peden
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, United Kingdom

P-027

PrP glycosylation-independent amplification of prions using highly efficient cell-based
protein misfolded cyclic amplification
Mohammed Moudjou
INRA, UR892, Virologie Immunologie Moleculaires, Jouy-en-Josas, France

P-028

Validating human stem cell derived neural cultures as a flexible model system in which
to investigate neurodegenerative mechanisms
James D Alibhai
The National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, The University of Edinburgh,
United Kingdom

P-029

sCJD prion seeding activity in human urine by RT-QuIC
Gabriele Piconi
National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK

P-030

Comparison of the in vitro amplification efficiency of UK iatrogenic and sporadic
Creutzfeldt-Jakob disease subtypes by protein misfolding cyclic amplification
Marcelo Barria Matus
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, The University of Edinburgh, United
Kingdom

P-031

Determination of the amino terminal domains of the cellular prion protein that are
required for highly efficient prion propagation
Parineeta Arora
MRC, United Kingdom

P-032

Prion-type dependent deposition of PRNP allelic products: Study in scrapie and BSE
infected heterozygous ARR/VRQ sheep
Jan PM Langeveld
Central Veterinary Institute part of WageningenUR, Netherlands

P-033

PrP quaternary structure and prion capacity to cross the species barrier
Angelique Igel
INRA UR892 VIM, France

P-034

Influence of a polymorphism in the highly conserved hydrophobic core region on
chronic wasting disease prion propagation and pathogenesis
Samia Hannaoui
University of Calgary, Faculty of Veterinary-Medicine, Dept. of Ecosystem and Public Health, Canada- 30 -

P-035

From misfolding to aggregation: Sequence effects on conformational properties ofamyloidogenic peptides implicated in neurodegeneration
Nikolay Blinov
National Institute for Nanotechnology, Canada; Department of Mechanical Engineering, University of Alberta, Alberta,
CanadaP-036

Modulation of protein quality control pathways as a novel intervention strategy inprion diseases
Simrika Thapa
Department of Comparative Biology and Experimental Medicine, Faculty of Veterinary Medicine, University of Calgary,
CanadaP-037

Effect of substitutions equivalent to bank vole 109I polymorphism in the spontaneous
misfolding ability of PrPs from several mammalian species
Hasier Erana
CIC bioGUNE, Parque Tecnologico de Bizkaia, Derio, Spain

P-038

Differences in the denatured state of wild-type and E211K bovine PrP
Eric M Nicholson
USDA / Agricultural Research Service / National Animal Disease Center, United States

P-040

Cellular phenotypes of prion disease in skin-derived fibroblasts of asymptomatic PrP
mutation carriers and sporadic CJD patients
Wenquan Zou
Case Western Reserve University, Pathology, United States

P-041

Gerstmann-Sträussler-Scheinker diseases with P102L, A117V and F198S mutationstransmit efficiently and produce distinct pathological phenotypes in bank voles
Laura Pirisinu
Istituto Superiore di Sanità, Dept. of Veterinary Public Health and Food Safety, Italy

P-042

Identification of the origin of Creutzfeldt-Jakob disease after cadaver-sourced pituitary
growth hormone treatment using an amplification property in protein misfolding
cyclic amplification
Atsuko Takeuchi
Department of Neurological Science, Tohoku University Graduate School of Medicine, Japan

P-043

Curing PrP-sc by a gene-interference strategy targeting biosynthesis of undersulfatedheparan sulphateDavid Cullis-Hill
Sylvan Scientific Pty Ltd., Australia

P-044

Kinetics of RML prion propagation in three inbred mouse strains with indistinguishable
expression levels of PrPC but distinct incubation periods
Malin K Sandberg
MRC Prion Unit, Department of Neurodegenerative Disease, United Kingdom

P-045

A cellular bioluminescence assay detects prion protein dimerization and aggregation
upon infection
Gültekin Tamgüney
German Center for Neurodegenerative Diseases (DZNE), Germany

P-046

Altered dynamics of membrane microdomain distribution in chronically prion-infectedcellsPeter C. Kloehn
UCL Institute of Neurology, MRC Prion Unit, United Kingdom

P-047

A Comprehensive Study of The Potential Resistance of The Canidae Family to Prion
Infection
Natalia Fernandez-Borges
CISA-INIA, Carretera de Algete a El Casar s/n, Valdeolmos, Madrid, Spain; CIC bioGUNE, Derio, Bizkaia, Spain- 31 -

P-048Classification of anti-prion compounds based on the binding properties to prion
proteins
Yuji O. KamatariLife Science Research Center, Gifu University, Japan

P-049

A platinum compound targeting the cysteine residues of disease-related form of prion
protein in cell lysates
Yuji Sakasegawa
Department of Neurochemistry, Tohoku University Graduate School of Medicine, Japan

EP-004Single Molecule Studies of Protein Aggregates in Prion Diseases
Chieh Sang
Department of Chemistry, University of Cambridge, United Kingdom

EP-005

Real-time quaking-induced conversion analysis for the diagnosis of sporadic
Creutzfeldt-Jakob disease in Korea
Jeong Ho Park
Ilsong Institute of Life Science, Hallym University, Korea; Korea CJD Diagnostic Center, Hallym University

EP-006

Diffusion-weighted images in patients with dura mater graft-associated CreutzfeldtJakob disease
Kenji Sakai
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science,Japan

EP-007

Post-translational modifications in the prion protein determine disease outcome
Christina Sigurdson
Pathology, University of California, United States

EP-008A self-propagating protease-resistant baculovirus-derived recombinant prion protein
is spontaneously generated by protein misfolding cyclic amplification at hightemperature
Morikazu Imamura
National Institute of Animal Health, Japan

EP-009Emergence and Host Dependent Propagation of Chronic Wasting Disease strains
Camilo DuqueCenter for Prions and Protein Folding Diseases, Canada

P-050

A kinetic model of the aggregation of alpha-synuclein provides insights into prion-likespreadingMarija Iljina
Chemistry Department, University of Cambridge, United Kingdom

P-051

Proteomic screening and identification of a structural protein of [NSI+] prion
determinant in yeast Saccharomyces cerevisiae
Anton A. Nizhnikov
Dept. of Genetics and Biotechnology, St. Petersburg State University; St. Petersburg Branch, Vavilov Institute of General
Genetics, Russian Academy of Sciences, Universitetskaya nab, St. Petersburg, Russia

P-052

Aggregation of QN-rich fragment of Gln3 in yeast Saccharomyces cerevisiae ismodulated by [PSI+] and [PIN+] prions
Kirill S. Antonets
Dept. of Genetics and Biotechnology, St. Petersburg State University, Russian Federation; St. Petersburg Branch, Vavilov
Institute of General Genetics, Russian Academy of Sciences

P-053

Prion-like Proteins and Disease Propagation in ALS
Neil R Cashman
Neurology, University of British Columbia, Canada- 32 -

P-055

Yeast-based Search for New Human Amyloidogenic Proteins
Nina V. Romanova
St Petersburg State University, St Petersburg, Russia

P-056

Analysis of Interspecies Prion Transmission in Yeast
Aleksandr A. Rubel
St Petersburg State University, St Petersburg, Russia

P-057

Formation of a metastable stress-inducible prion by the yeast short-lived actin
associated protein
Tatiana A. Chernova
Department of Biochemistry, Emory University School of Medicine, Atlanta, USA

P-058

Emergence and evolution of prion and prion-like proteins in the eukaryotic domain
Paul M Harrison
McGill University, Dept. of Biology, Canada

P-059

Latent structural variation in a yeast prion monomer determines strain phenotypes
Motomasa Tanaka
RIKEN Brain Science Institute, Japan

P-060

Chaperone sorting factor Cur1 exhibits differential effects on yeast prions
Galina A. Zhouravleva
Dept. of Genetics and Biotechnology, St Petersburg State University, Russian Federation; Laboratory of Amyloid Biology,
St Petersburg State University

P-061

Modulation of Yeast Prion Strain Competition by Host Genetic Background andMolecular Chaperons
Chang-I Yu
Institute of Molecular Biology, Academia Sinica, Taipei, Taiwan, R.O.C.; Department of Life Sciences and Institute of
Genomic Sciences, National Yang-Ming University, Taipei, Taiwan, R.O.C.

P-062

Prophylactic efficacy of orally administered compounds on the progression of scrapie
induced motor coordination deficits
Damani N Bryant
Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, United States

P-063

We shall overcome prion diseases only by using both scientific and empiric findings
or why honoring discovery and development of avermectins with 2015 Nobel Prizefor physiology or medicine does not decrease their chronic toxicity especially neuraldegeneration increasing the susceptibility for prion diseases
Andreas Becker
Independent Institute for Holistic Prion Research, Germany

P-064

Low activity of complement in the cerebrospinal fluids of the patients with various
prion diseases
Cao Chen
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control andPrevention, Chinese Center for Disease Control and Prevention, China; Collaborative Innovation Center for Diagnosis
and Treatment of Infectious Diseases, Zhejiang University

P-065

Myelin basic protein citrullination as predictive marker of demyelination contributes
to the pathogenesis of prion diseases
Byungki Jang
Ilsong Institute of Life Science, Hallym University, Anyang, Korea

P-066

Emergence of two prion subtypes in ovine PrP transgenic mice infected with human
MM2-cortical Creutzfeldt-Jakob disease prions
Vincent Beringue
INRA, France- 33 -

P-067The density of M cells in the epithelium overlying the Peyer's patches influences
susceptibility to oral prion disease
Neil A. Mabbott
The Roslin Institute & R(D)SVS, University of Edinburgh, UK

P-068

Challenging the central hypothesis that misfolded prion protein accumulation, spreadand distribution predicts regions of neurodegeneration
James D Alibhai
The Roslin Institute, The University of Edinburgh, United Kingdom

P-069

Transcriptome analysis of microglia and astrocytes in prion-infected mice
Minori Kuroda
Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University, Japan

P-070

Transmission properties of human PrP 102L prions challenge the relevance of mousemodels of GSS
Emmanuel A Asante
MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for
Neurology and Neurosurgery, Queen Square, London, United Kingdom

P-071

Impairment of protease activated receptors calcium signaling in prion infected celllines
Tibor Mosko
Institute of Immunology and Microbiology, First Faculty of Medicine, Charles University in Prague, Czech Rep.

P-072

Defining the Microglia Response during the Time Course of Chronic Neurodegeneration
Jean C Manson
University of Edinburgh, United Kingdom

P-073

The route of inoculation of kuru during traditional mortuary feasts in the kuruaffected region
Jerome T Whitfield
MRC Prion Unit, United Kingdom

P-074

New insights in the transfusional risk assessment of variant Creutzfeldt-Jakob Disease:
Transfusional transmission of vCJD prions in the absence of detectable abnormal prion
protein
Emmanuel E Comoy
CEA DRF/iMETI/SEPIA, Fontenay-aux-Roses, France

P-075

Neuronal toxicity of the expression of human PrP with the E200K mutation in themecanosensitive neuronal system of the nematode C. elegans
Bizat M. Nicolas
INSERM; INSERM UMRS 1127, CNRS UMR 7225, UPMC, Institut du Cerveau et de la Moelle Epiniere, G.H. PitieSalpetriere, Paris, France; Université Paris Rene Descartes, Paris, France

P-076

Impact of human prion proteins with E211D or E211Q mutation on tau alterations in
cultured cortical neurons
Alexianne Gougerot
Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière (CRICM),UMRS 975, Equipe Maladie d'Alzheimer - Maladies à Prions, Paris, France; Centre National de Reference des Agents
Transmissibles Non Conventionnels (InVS), France

P-077

Is sporadic CJD an acquired disease? A review of the UK CJD cases
Patrick JM Urwin
National CJD Research & Surveillance Unit, United Kingdom

P-078

Subcutaneous Administration of Mouse Recombinant Prion Protein Resistant or RML
Trigger Long-term Alterations in Composition of Intestinal Microbiota in FVB/N Female
Mice
Burim Ametaj
University of Alberta, Dept. of Agricultural, Food and Nutritional Science, Canada- 34 -

P-079Effect of scrapie infection on expression of endogenous retroviral genes in sheep
lymphoid tissues
Fiona Houston
The Roslin Institute, United Kingdom

P-080

Early Age Oral Administration of Mouse Recombinant Prion Protein Resistant or RML
Trigger Lifelong Modifications in the Composition of Intestinal Microbiota in FVB/N
Female Mice
Suzanna M Dunn
University of Alberta, Department of Agricultural, Food and Nutritional Science, Canada

P-081

Importance of Complement Receptor CD21 in Establishing Peripheral Prion
Accumulation
Eric M Swanson
Colorado State University, United States

P-082

Infectious CWD prions detected at the feto: Maternal interface of experimental andfree-range naturally-exposed cervids
Candace K Mathiason
Microbiology, Immunology and Pathology, United States

P-083

Prion strain-dependent effect of macroautophagy on abnormal prion protein
degradation
Daisuke Ishibashi
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences,
Japan

P-084

Differential effects of anti-prion treatments highlight differences in the pathogenesisof sporadic and familial forms of prion disease and identifies novel mode of action for
pentosan polysulfate
Victoria A Lawson
Department of Pathology, The University of Melbourne, Australia

P-085

Withdrawn

P-086

Neuropathological analysis of hyperintense signals on magnetic resonance imaging in
MM1+2 type sporadic Creutzfeldt-Jakob disease
Ayano Shima
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences,
Japan

P-087

HDAC6 alleviates prion peptide-mediated neuronal death via modulating PI3K-AktmTOR pathway
Lifeng Yang
National Animal Transmissible Spongiform Encephalopathy Laboratory, China Agricultural University, Beijing, China

P-088

Transmission of experimental CH1641-like scrapie to bovine PrP overexpression mice
Kohtaro Miyazawa
Influenza and Prion Disease Research Center, National Institute of Animal Health, NARO, Japan

EP-014Progranulin: Potential regulator in pathogenesis of Prion disease?
Hee-Young Lim
Hallym University, Ilsong Institute of Life Science, Korea

EP-015

Pathognomonic inter-tissue transcriptome analysis following prion spread byintraperitoneal infection
Yeong-Gon Choi
Ilsong Institute of Life Science, Hallym University, Korea- 35 -

EP-016

Characterization of newly established neuronal cell lines from Bank Vole prion proteintransgenic miceHong-Seok Choi
Ilsong Institute of Life Science, Hallym University, Korea

EP-017Comparison of abnormal isoform of prion protein in prion-infected cell lines andprimary cultured neurons by PrPSc-specific immunostaining
Misaki Tanaka
Laboratory of Veterinary Hygiene, Graduate School of Veterinary Medicine, Hokkaido University, Japan

EP-018RNA editing: A novel approach to understand CJD pathogenesis
Eirini Kanata
Prion Diseases Research Group, School of Health Sciences, Department of Pharmacy, Aristotle University ofThessaloniki, Greece

EP-019Interspecies transmission of atypical L-BSE prion to non-human primates (cynomolgus
macaques) alleviates PrPSc glycoform profile of cattle L-BSE trait, but preservesincompetent transmissibility to inbred mice
Ken'ichi Hagiwara
Department of Biochemistry and Cell Biology, National Institute of Infectious Diseases, Japan

P-089

New CSF-based approaches in the differential diagnostic of sCJD
Franc Llorens
German Center for Neurodegenerative Diseases, Germany; Department of Neurology, Clinical Dementia Center,
University Medical Center Gottingen, Germany

P-090

Identification of microRNA signature in sCJD reveals massive regional and subtypedependent regulation
Thüne Katrin
Department of Neurology, Medical University Göttingen, Germany; German Center for Neurodegenerative Diseases(DZNE)

P-091

Variant CJD: Lessons in Public Health
Abigail B Diack
The Roslin Institute, University of Edinburgh, United Kingdom

P-092

Identification of new molecular alterations in Fatal Familial Insomnia
Isidro Ferrer
University of Barcelona; Institute of Neuropathology, Bellvitge University Hospital, Hospitalet de Llobregat, Spain;
CIBERNED, Institute Carlos III, Spain

P-093

An autopsy case of MM1-type sporadic Creutzfeldt-Jakob disease with 1-month total
disease duration presenting with early disease pathology
Yasushi Iwasaki
Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Japan

P-094

Familial Creutzfeldt-Jacob disease (V180I) as the initial presenting depression:
Case report
Dooeung Kim
Neurology, Veterans Hospital, Seoul Medical Center, Korea

P-095

A survey of anti-prion compounds using the real time-quaking induced conversion (RTQuIC)
Jae Wook Hyeon
National Institute of Health (Korea CDC), Korea

P-096

Proteomics Analyses for the Global Proteins in the Brain Tissues of Different Human
Prion Diseases
Qi ShiPrion Department, National Institute for Viral Disease Control and Prevention, China CDC, China- 36 -

P-097

Genetic Creutzfeldt-Jacob disease with V180I mutation in Korea
Jae W Kim
Dong-A University, Korea

P-098

Genome differences between genetic Creutzfeldt-Jakob Disease's patients with V180I
mutation and healthy individuals and associations with other neurodegenerative
disorders
Sol Moe Lee
Division of Zoonoses, Center for Immunology & Pathology, National Institute of Health, Korea Centers for Disease
Control & Prevention, Korea

P-099

A Therapeutic Approach for Creutzfeldt-Jakob Disease by DNAzyme-mediatedKnockdown of the Prion Protein
Julian Victor
Institut für Physikalische Biologie, Heinrich-Heine-Universität Düsseldorf, Germany

P-100

Differential association of amyloid-β with PrPSc pathology in each genetic priondiseaseFumiko Furukawa
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Tokyo, Japan

P-101

Withdrawn

P-102

Brain Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) and
Neuropathologic Correlations in Human Prion Diseases
Brian S Appleby
Case Western Reserve University, National Prion Disease Pathology Surveillance Center, United States

P-103

Celia´s encephalopathy: A new member of the group of protein misfolding-mediatedneurodegenerative diseases
Jesús R. Requena
University of Santiago de Compostela, Spain

P-104

AR-12 and its derivatives, a potential new therapeutic agent against prions
Hermann M Schatzl
University of Calgary, Comp. Biol. & Exp. Med., Canada

P-105

An autopsy-verified case of FTLD-TDP with upper motor neuron predominant motor
neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease
Yuichi Hayashi
Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Japan

P-106

Temporal resolution of PrPSc transport, PrPSc accumulation, activation of glia andneuronal death in retinas from C57Bl/6 mice inoculated with RML scrapie: Relevance tobiomarkers of prion disease progression
M. Heather West Greenlee
Biomedical Sciences, Iowa State University College of Veterinary Medicine, United States

P-107

CSF analysis of patients with human prion disease
Katsuya Satoh
Department of Locomotive Rehabilitation Science, Unit of Rehabilitation Sciences, Nagasaki University Graduate Schoolof Biomedical Sciences, Japan

P-108

Prospective surveillance data of human prion disease in the Chugoku and Shikoku
regions of Japan
Kota Sato
Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences,Japan- 37 -

P-109Tau / p-Tau and the altered regulatory response of Rab7a contributing the fast
progression rate in Creutzfeldt-Jakob disease (CJD) and rapid progressive Alzheimer's
disease (rpAD)
Saima Zafar
Department of Neurology, Clinical Dementia Center and DZNE, Georg-August University, University Medical Center
Goettingen (UMG), Germany

P-110

Detection of disease-specific PrP and infectivity in the blood of mice with preclinicalprion disease - implications and applications for public health
Elizabeth B Sawyer
MRC Prion Unit, United Kingdom

P-111

Iatrogenic Creutzfeldt-Jakob disease in human growth hormone recipients in the
United Kingdom
Mark W HeadNational CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, United Kingdom

P-112

Assessing the disease-modifying role of TREM2 in a prion model of neurodegeneration
Jean C Manson
The Roslin Institute, United Kingdom

P-113

Two International Ring-trials demonstrate that CSF RT-QuIC is a robust and reliable testfor diagnosing sporadic CJD
Neil I. McKenzie
University of Edinburgh, National CJD Research and Surveillance Unit, Western General Hospital, Edinburgh, United
Kingdom

P-114

Enhanced Creutzfeldt-Jakob disease surveillance in the older population in the UK:
Biochemical analysis for PrPSc
Helen M Yull
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, UK

P-115

Accuracy of Creutzfeldt-Jakob disease diagnosis using RT-QuIC testing of nasal andcerebrospinal fluid samples
Gianluigi Zanusso
University of Verona, Department of Neurosciences, Biomedical and Motor Sciences, Italy

P-116

A novel approach combining 3D human cell culture and 3D microscopy to assess prion
infectivity
Ferid Nassor
CEA DRF/iMETI/SEPIA, Fontenay-aux-Roses, France

P-117

Iatrogenic CJD after human GH treatment in France: Effect of sex, dose and genetics
on the susceptibility of a possible infection by a V2 sCJD strain
Laurene Peckeu
Team Alzheimer's and Prion Diseases, Inserm UMR-1127/CNRS UMR 7225, Université Pierre & Marie Curie - Paris 6,
FranceP-118

Volumetric analysis and Diffusion Tensor Imaging in Creutzfeldt-Jakob disease andfatal insomnia point to the thalamus as a key structure in disease pathogenesis
Oriol Grau-Rivera
Neurological Tissue Bank of the Biobanc-Hospital Clinic-IDIBAPS, Spain

P-119

Prion protein interactome: Identifying novel targets in rapidly progressive Alzheimer's
diseaseMohsin Shafiq
Clinical Dementia Center, Department of Neurology and Psychiatry, University Medical Center Goettingen, Germany

P-120

Assessment of doxycycline treatment on prion deposition in the olfactory epitheliumof patients with Fatal Familial Insomnia: Possible mirroring of the CNS alterations
Fabio Moda
IRCCS Foundation Carlo Besta Neurological Institute, Department of Neuropathology and Neurology, Italy- 38 -

P-121

Unusually young prion disease cases in the United States, 1979-2014
Ryan A. Maddox
National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC), USA

P-122

End-Point Quaking-Induced Conversion (EP-QuIC) as a Routine Test for sCJD in Canada
J. David Knox
Public Health Agency of Canada, Canada; Department of Medical Microbiology, University of Manitoba

P-123

Rapid testing for Creutzfeldt-Jakob disease in donors of human tissues
David M. Asher
US Food and Drug Administration, Center for Biologics Evaluation and Research, United States

P-124

Rational design and optimization of drug leads targeting prion-like misfolding andaggregation of SOD1 enzyme in Amyotrophic Lateral Sclerosis
Vijaya Kumar Hinge
National Institute for Nanotechnology, Canada; Department of Mechanical Engineering, University of Alberta, Alberta,
CanadaP-125

Novel detection technique of early Alzheimer's disease from blood using fluorescence
spectral microscopy
Shigeki Tsutsui
Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, Canada

P-126

Geographic risk of variant Creutzfeldt-Jakob disease: A risk ranking model to evaluate
options for blood donor deferral policies in the US
Hong Yang
US Food and Drug Administration, United States

P-127

Early response of Cofilin1 pathway in Creutzfeldt Jakob disease
Neelam Younas
Department of Neurology, Clinical Dementia Center and DZNE, Georg-August University, University Medical Center
Goettingen (UMG), Germany

P-128

Clinical courses of patients with Creutfeldt-Jakob disease in Shizuoka Institute of
Epilepsy and Neurological Disorders, Japan
Tomokazu Obi
Shizuoka Institute of Epilepsy and Neurological Disorders, Department of Neurology, Japan

P-129

Epidemiologic features of human prion diseases in Japan: A prospective 15-year
surveillance study
Ryusuke AeDivision of Public Health, Center for Community Medicine, Jichi Medical University, Japan

P-130

Diagnostic significance of Periodic synchronous discharges in Japanese surveillance ofCreutzfeldt-Jakob disease
Yoshiyuki Kuroiwa
Teikyo University Mizonokuchi Hospital, Japan

P-131

Creutzfeldt-Jakob disease associated with a V203I homozygous mutation in the prion
protein gene
Junji Komatsu
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences,
Japan

P-132

Clinical features in the patients with V180I, M232R and P102L of PRNP
Erika Abe
Neurology, National Hospital Organization Akita Hospital, Japan

P-133

Suspected prion disease cases referred to the National Prion Disease Pathology
Surveillance Center, United States
Ermias D Belay
Centers for Disease Control and Prevention, Atlanta, Georgia, United States- 39 -

P-134

CSF biomarkers, Tau and 14-3-3, in genetic and sporadic Creutzfeldt-Jakob disease
patients in Israel
Zeev Meiner
Physical Medicine and Rehabilitation, Hadassah-Hebrew University Hospital, Israel

P-135

The Japanese Consortium of Prion Disease (JACOP) for patients’ registration andclinical studies of Prion diseases in Japan
Yuko Ishimura
National Center Hospital, National Center of Neurology and Psychiatry, Japan

P-136

Cerebral beta-amyloidosis in patients with dura mater graft-associated CreutzfeldtJakob disease
Tsuyoshi Hamaguchi
Department of Neurology, Kanazawa University Hospital, Japan

P-137

Withdrawn

P-138

Clinically and neuropathologically atypical autopsied case of sporadic CreutzfeldtJakob disease MM type1
Yuta Nakano
Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institution of Gerontology, JapanP-170

CJD International Support Alliance (CJDISA) - The voice, the face, the human story
behind this horrific disease
Suzanne Solvyns
CJD International Support Alliance, Australia; CJD Support Group Network Australia; CJD Insight; CJD Foundation USA;CJD Support Network Japan; CJD Support Network UK; A.I.En.P. Italy; MCJ-HCC France; CJK Initiative e.V. Germany;CJD Foundation Israel

P-139

How can we increase the number of prion autopsy in Japan?
Masaki Takao
Saitama International Medical Center; Mihara Memorial Hospital, Japan

P-140

Wire-QuIC: A new detection system of human prion
Tsuyoshi Mori
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences,
Japan

P-141

The first Italian case of Creutzfeldt-Jakob disease with V180I mutation in the PrP gene
(PRNP)
Maurizio Pocchiari
Istituto Superiore di Sanità, Cell Biology and Neurosciences, Italy

P-142

Evaluation of CSF RT-QuIC diagnostic assay for Creutzfeldt-Jakob and other humanprion diseases: The Italian Surveillance Unit experience
Maurizio Pocchiari
Istituto Superiore di Sanità, Cell Biology and Neurosciences, Italy

P-143

A case of slowly progressive familial prion disease with a five-octapeptide repeat
insertion
Makoto Takahashi
Department of Neurology, Kanto Central Hospital, Japan

P-144

Human Prion Diseases Surveillance and Registration System in Japan
Tadashi Tsukamoto
Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Japan

P-171

Presymptomatic genetic testing for genetic prion disease: What should we consider
and how should we deal with it?
Chieko Tamura
FMC Tokyo Clinic, Japan; Genetic Counseling Clinic, Juntendo University Hospital- 40 -

EP-022

Agraphia of Kanji (Chinese characters): An early symptom of sporadic CreutzfeldtJakob disease in a Japanese patient
Keiko Nakamura
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences,Japan

EP-023

Establishment of high-sensitivity detection method of FABP3 in cerebrospinal fluid of
CJD patients
Yumi Tanaka
Department of Molecular and Applied Biosciences, Hiroshima University Graduate School of Biosphere Science, Japan

EP-024

Annual incidence of Gerstmann-Stäussler-Scheinker disease in Kyushu region of Japan
Yoshio Tsuboi
Department of Neurology, Fukuoka University, Japan

EP-025Generation of Alzheimer's disease (AD) genetic patients reprogrammed stem cells (iPS)
as tools for the study of AD physiopathology
Laura Auboyer
Institute for Regenerative Medicine and Biotherapy (IRMB), France

EP-026

SPECT study of the Nigrostriatal Dopaminergic System in Creutzfeldt-Jakob disease:
A case report and literature review
Tzu-Hsuan Chen
Department of Physical Medicine and Rehabilitation,Shin Kong Wu Ho-Su Hospital, Taipei, Taiwan

EP-027

Epidemiological survey of Gerstmann-Straussler-Scheinker disease with codon 102
mutation in Japan
Hiroyuki Murai
Department of Neurological Therapeutics, Kyushu University, Japan

EP-028A case of subacute progressive dementia presenting with M129V mutation of theprion protein gene and positive RT-QUIC assays
Rie Motoyama
Neurology, Tokyo Metropolitan Geriatric Hospital, Japan

EP-029Inhibition of The interaction between Prion Protein and Amyloid beta protein byComputational Methods; A strategy Against Alzheimer's Disease
Michael K Fonjang
German School for Simulation Science (GRS), Cameroon

P-145

Estimating chronic wasting disease resistance in cervids using real time quakinginduced conversion
Nicholas J Haley
Department of Microbiology and Immunology, Midwestern University, United States

P-146

RT-QUIC detection in CWD infected cervid and TgElk mice tissues
Hyun Joo Sohn
Animal and Plant Quarantine Agency (QIA), Korea

P-147

Infection and detection of PrPCWD in soil from CWD infected farm in Korea
Hyun Joo Sohn
Animal and Plant Quarantine Agency (QIA), Korea

P-148

Prion treatment by electrolyzed alkaline water and other chemicals
Takashi Onodera
University of Tokyo, JapanP-149

Key Features of the Australian BSE Food Safety Assessment Process
Hong Jin
Food Standards Australia New Zealand, Australia- 41 -

P-150Study of the allelic variants at codon 222 in the genome goat for the detection ofsusceptibility to SCRAPIE, through the mass spectrometry (MALDI-TOF)
Daniele Macri'
Istituto Zooprofilattico Sperimentale della Sicilia, Italy

P-151

Phenotypic plasticity of chronic wasting disease prions
Christina M Carlson
U.S. Geological Survey National Wildlife Health Center, United States

P-152

Sensitive and Rapid Diagnosis of Goat Prion Diseases by Real Time Quaking Induced
Conversion Assay
Alessandra Favole
Istituto Zooprofilattico Sperimentale del Piemonte Liguria e Valle d'Aosta, Italy

P-153

Experimental oral transmission of chronic wasting disease to sika deer (Cervus nippon)
Gordon Mitchell
National & OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario,
CanadaP-154

Prion infectivity detected in swine challenged with chronic wasting disease via theintracerebral or oral route
S Jo Moore
Virus and Prion Research Unit, National Animal Disease Center, ARS, USDA, United States

P-155

Oral Transmission of Classical BSE to Adult Cattle
Sandor Dudas
Canadian and OIE BSE Reference Laboratory, Canadian Food Inspection Agency, National Center for Animal Disease
Lethbridge, Canada

P-156

Detection of Prion Protein Specific Camelid Nanobodies: Implications for Prion Disease
Therapeutic Options
Savannah M Rocha
Colorado State University, United States

P-157

Earthworms can act as carriers for prion disease transmission
Sandra Pritzkow
University of Texas Health Science Center at Houston, Germany; Mitchell Center for Alzheimer's Disease and Related
Brain Disorders

P-158

Prion Protein Gene Sequences Analysis in Twelve Sheep Breeds of Pakistan
Mohammad Farooque Hassan
China Agricultural University, Beijing, China; SBBUVAS, Sakrand Sindh, Pakistan

P-159

The epidemiological evolution of prion infection on bovine in Romania, in the periodof 2010 - 2015
Florica Barbuceanu
Institute for Diagnosis and Animal Health, Romania; Faculty of Veterinary Medicine Bucharest

P-160

Rapid tests might overlook bovine spongiform encephalopathy infection in goats
Daniela Meloni
CEA, Istituto Zooprofilattico Sperimentale dl Piemonte, Liguria e Valle d'Aosta, Turin, Italy

P-161

A high incidence of atypical scrapie in a closed flock of Cheviot sheep
Fiona Houston
Neurobiology Division, The Roslin Institute, University of Edinburgh, United Kingdom

P-162

Local and Traditional Knowledge in Monitoring of Chronic Wasting Disease andWildlife Health in Western Canada
Brenda L Parlee
Dept. Resource Economics & Env. Sociology, Faculty of Agricultural, life and Environmental Sciences, University ofAlberta, Canada- 42 -

EP-010

Simplified Rapid Detection of Prions in Biological Fluids Using Particle Extraction andReal-time Quaking Induced Conversion
Nathaniel D Denkers
Colorado State University, Dept. Microbiology, Immunology, and Pathology, United States

EP-011

Neuronal death and prion diseases
Corinne I Lasmezas
Department of Immunology and Microbial Science, Department of Neuroscience, The Scripps Research Institute,
United States

EP-012

Delipidation of brain tissue partially removed the inhibitory effect of prion amyloid
formation
Yoshifumi Iwamaru
National Institute of Animal Health, Japan

EP-013

Withdrawn

P-163

Active vaccination against chronic wasting disease using multimeric rec-PrP:
A promising approach to contain CWD
Dalia HA Abdelaziz
Department of Comparative Biology & Experimental Medicine, Faculty of Veterinary Medicine, University of Calgary,Alberta, Canada

P-164

Adsorption of Soluble Prions by Metals Is Limited by Plasma
Maurizio Pocchiari
Istituto Superiore di Sanità, Italy

P-165

CJD incidents in Japan
Ichiro Takumi
Department of Neurosurgery, Nippon Medical School Musashi Kosugi Hospital, Japan; Japan CJD Indecent Committee

P-166

Inactivation of atypical and classical BSE prions by laboratory-scale autoclaving
James Hope
Animal and Plant Health Agency, United Kingdom

P-168

Three-dimensional cultures of murine neurones demonstrate prion-induced plaquepathology and cell death
Cathryn L Haigh
Department of Medicine, Royal Melbourne Hospital, The University of Melbourne, Australia

P-169

Building a Program for Community-Based Monitoring of Wildlife Health - Lessons for
Surveillance of Chronic Wasting Disease in Moose and Deer in Western Canada
Brenda L. Parlee
Dept. of Resource Economics and Environmental Sociology, Faculty of Agricultural, life and Environmental Sciences,
University of Alberta, Canada

EP-020Prion seeding activity is widely distributed in tissues of sporadic Creutzfeldt-Jakob
disease patients
Katsuya Satoh
Department of Locomotive Rehabilitation Science, Nagasaki University Graduate School of Biomedical Sciences, Japan

EP-021

Canine Prions: A New Form of Prion Disease
Mourad Tayebi
University of Melbourne, Veterinary Sciences, Australia- 43 -