Scientific Programme  


Preliminary Scientific Programme (Venues to be confirmed)

The programme of PRION 2008 will include all contemporary topics in Prion Disease.

Scientific sessions will be organised covering all the important subjects in the TSE field.

Topics

  • Basic mechanics of neurodegeneration: Healthy and degenerating neurons
  • Diagnostics
  • Emergent strains
  • Epidemiology
  • Genetics
  • In vitro and in vivo replication
  • Prion transmission and pathogenesis
  • Protein misfolding in neurodegenerative disease
  • Risk assessment
  • The functions and cell biology of PrPc
  • Therapeutics
  • Yeast prions
  • IFPA session: Progress with blood safety and prions

Menu

Tuesday 7th October 2008

08:30-09:30. Registrations
Auditorium Hotel
09:30 –15:00. Workshop: “TSEs in goats, cervids and sheep”
Auditorium Hotel(Roma Room)
Chair: Dr. Jan Langeveld
10.00 - 13.00. Public TSE Lecture: “Scrapie en oveja”
INIA (Salon de Actos). Carretera de la Coruńa Km 7,5
Chairs: Dr. Juan Maria Torres, Dra. Malena Serrana
13.00 - 19.00. Afternoon Satellite Session: "Risk Assessment and Management Event"
Hotel Auditorium – Frankfurt and Munich Rooms
Avenida de Aragón, 400, 28022 Madrid, Spain
16.00 - 19.00. Public TSE Lecture: “Encefalopatias Espongiformes Humanas”


Información adicional:jpedro@isciii.es


Wednesday 8 October 2008

Horario Auditorium Horario Roma room
8:45-9:15
Opening Ceremony

  • Dr. Cristina Garmendia, Minister of Science and Innovation of the Spanish Government.
  • Dr. Juan-Maria Torres, CISA-INIA Madrid (Congress Chairman)
  • Dr. Jean-Phillipe Deslys, NeuroPrion.
    		•   
    9:15-9:45 (S1) Plenary conference:
    "The secret life of prion strains"
    Dr. Charles Weissmann
    9:50-11:10 (1) Session 1. Basic mechanism of neurodegeneration
    Chair: Jean Manson, Francisco Wandosell    		 10:00-13:00 (4) Session 4. Epidemiology & risk assessment
    Chair: Jim Hope, Juan Badiola
    9:50-10:10 (OC1.1) Mutant Prion Protein Expression Causes Motor and Memory Deficits and Abnormal Sleep Patterns in a New Transgenic Mouse Model
    		 09:50-10:10 (OC4.1) Comparative analysis of the trend of the BSE epidemic across Europe, in relationship with control measures
    10:10-10:30 (OC1.2) Identification of Neuron-specific gene Regulatory Circuits Involved in Prion-Induced Neurodegeneration
    		 10:10-10:30 (OC4.2) Increasing the age limit in active BSE surveillance to 48 months: How many cases will we miss?
    10:30-10:50 (OC1.3) Cyclin dependent kinase 5 phosphorylation induces prion protein conformational change.
    		 10:30-10:50 (OC4.3) Scrapie control through selective breeding in the Netherlands: when will it be achieved?
    10:50-11:20 Coffee break
    11:20-13:00 (2) Sesion 2. protein misfolding
    Chair: Holger Wille, Jesús Requena    		 11:20-13:00 (4) Session 4. Epidemiology & risk assessment (cont.)
    Chair: Jim Hope, Juan Badiola
    11:20-11:40 (OC2.1) Monitoring prion protein stability by NMR
    		 11:20-11:40 (OC4.4) A case control study on atypical scrapie in French sheep
    11:40-12:00 (OC2.2) Limited proteolysis of PK-sensitive PrPSc further supports a structure featuring beta sheet stretches interspersed with loops/turns for PrPSc
    		 11:40-12:00 (OC4.5) Diagnostic profile of young patients referred to the CJD Italian surveillance unit
    12:00-12:20 (OC2.3) Different strains have different “most infectious” particles
    		 12:00-12:20 (OC4.6) Human transmissible Spongiform Encefalopaties in Spain, 1993-2008.
    12:20-12:40 (OC2.4) In vivo transmission of Alzheimer’s disease by a prion like mechanism.
    		 12:20-12:40 (OC4.7) Combining Quantitative Models for BSE and vCJD: Developing an Accessible Tool for Prion Disease Risk Analysis
    12:40-13:00 (OC2.5) Infectious and non-infectious aggregates of the prion protein share several conformational epitopes
    		 12:40-13:00 (OC4.8) TSE Roadmap – Results of a comparative study of risk perception and risk communication of stakeholders within European countries
    13:00-14:20 Lunch / Poster Session
    14:20-15:00 (S2) Plenary conference:
    "Yeast prion structure explains how proteins can be genes".
    Dr. Reed Wickner    		  
    15:00-17:00 (3) Session 3. Prion transmission & pathogenesis
    Chair: Fabrizio Tagliavini, Martí Pumarola    		 15:00-16:20 (5) Session 5.The function & cell biology of PrP (4)
    Chair: David Harris, Toni del Rio
    15:00-15:15 (OC3.1) Transmission of atypical BSE to Microcebus murinus, a non-human primate: Development of clinical symptoms and tissue distribution of PrPres.
    		 15:00-15:20 (OC5.1) Association of cellular prion protein and sphingolipid-mediated signaling pathways
    15:15-15:30 (OC3.2) Oral transmission of BSE in resistant ARR/ARR sheep: involvement of lymphoid tissues
    		15:20-15:40 (OC5.2) Dose independent modulations of glutamate receptors expression and neuronal excitability by PrPc in vivo.
    15:30-15:45 (OC3.3) Prions hi-jack tunneling nanotubes for intercellular spread
    		 15:40-16:00 (OC5.3) Signalling and endocytosis: coupled mechanisms in cellular prion physiological functions
    15:45-16:00 (OC3.4) New hospital prion decontamination procedures using copper-hydrogen peroxide formulations with optimal chemical compatibility and reduced safety risks
    		 16:00-16:20 (OC5.4) Effect of disease-associated mutations in the prion protein on the beta-secretase cleavage of the amyloid precursor protein
    16:00-16:15 (OC3.5) Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay
    		 16:20-17:00 (6) Session 6 - Genetics
    Chair: Theo Sklaviadis, Cristina Acin
    16:15-16:30 (OC3.6) Neuroinvasion in sheep TSEs: the role of the haematogenous route
    		 16:20-16:40 (OC6.1) Transcriptional Regulation of Prion Protein Gene Expression by SP1 and MTF1
    16:30-16:45 (OC3.7) The Structure of Rabbit PrP-C: Clues into TSE fibril formation and the species barrier.
    		 16:40-17:00 (OC6.2) Cathepsin D C224T polymorphism in sporadic and genetic Creutzfeldt-Jakob disease
    16:45-17:00 (OC3.8) Prions in milk from ewes incubating natural scrapie
    		 17:00-17:20 (OC6.3) Investigation of protein-protein interactions from scrapie infected mice brain tissue using NAPPA
    17:00-17:15 (OC3.9) TSE neuroinvasion is dramatically impaired in aged mice
    		  
    17:20-20:00 Welcome Reception/Poster Party

    Thursday 9 October 2008

    Horario Auditorium Horario Roma room
    9:00-09:30 (S3) Plenary conference
    "A mouse model of fatal familial insomnia"
    Dr. Susan Lindquist    		  
    9:30-13:00 (3) Session 3. Prion transmission & Pathogenesis (cont.)
    Chair: Fabrizio Tagliavini, Martí Pumarola    		 9:30-13:00 (9) Session 9. Diagnostics
    Chair: Jan Langeveld, Tomás Mayora
    9:50-10:10 (OC3.10) Reversible changes of PrPSc after transmission in animals with mismatched prion proteins
    		 09:30-09:50 (OC9.1) BSE / Scrapie intracerebrally co-infected sheep: Scrapie hides BSE in the brain, not in lymphoid tissues
    09:45-10:00 (OC3.11) Strain targeting without strain-specific axonal transport or neuronal tropism
    		 09:50-10:10 (OC9.2) Sensitive Biomarker Detection of Protein Folding Disorders; Proximity Ligation-based detection of protein Oligomers
    10:00-10:15 (OC3.12) vCJD in primate: new insights for blood risk assessment
    		 10:10-10:30 (OC9.3) The Identification of Biomarkers in the Urine of BSE Infected Cattle
    10:15-10:30 (OC3.13) The Sup35p NM domain propagates as a prion in mammalian cells
    		 10:30-10:45 (OC9.4) Protein misfolding cyclic amplification combined with the conformation dependent immunoassay as a potential confirmatory blood test for variant Creutzfeldt-Jakob Disease
    10:30-10:45 (OC3.14) The influence of PrP glycosylation for the spreading of the TSE infectious agent and in determining the strain characteristics.
    		 10:45-11:00 (OC9.5) High resolution quantification and differentiation of PrPSc forms in mixed BSE and scrapie samples by N-TAAP demonstrates potential for detection of co-infection.
    10:45-11:00 (OC3.15) The glycosylation of prion protein is not required for the prion strain formation
         		11:00-11:10 (OC9.6) Specificity and capacity of the P-Capt® filter for prion protein and prion infectivity
    11:10-11:40 Coffee break
    11:40-13:00 (7) Session 7. Emergent strains
    Chair: Maria Caramelli, Juan María Torres    		 11:40-13:00 IPFA Session:
    Progress With Blood Safety and Prions
    Chair: Robert Will, Paul Brown
    11:40-12:00 (OC7.1) BASE in sheep displays strain features distinct from BSE and scrapie
    		 11:40-12:05 Do sporadic or genetic CJD pose a risk through blood or blood products?
    12:00-12:20 (OC7.2) When Atypical Scrapie cross species barriers.
    		 12:05-12:20 Update on blood screening tests (yet again), with some ‘strategic’ comments
    12:20-12:40 (OC7.3) Mutational changes affecting thermostability of TSE agent-strains?
    		 12:20-12:35 Blood based assays for infection with the agent of vCJD.
    12:40-13:00 (OC7.4) North American Cervids Harbor Two Distinct CWD Strains
    		 12:35-13:00 Spiking for blood product validation
    13:00-14:20 Lunch / Poster Session
    14:30-15:00 (S4) Plenary conference:
    "Towards imaging prion replication and spread "
    Dr. Baron    		  
    15:00-18:00 (8) Session 8. In vitro and in vivo replicatio
    Chair: Ilia Baskakov, Joaquín Castilla     		15:00-18:00 (10) Session 10. Therapeutics
    Chair: Gianluigi Forloni, Pablo Martínez
    15:00-15:20 (OC8.1) In vitro amplification of PrPSc derived from brain and blood of sheep infected with scrapie
    		 15:00-15:20 (OC10.1) An Update on the First U.S. Treatment Trial for Sporadic Jakob-Creutzfeldt Disease (sCJD)
    15:20-15:40 (OC8.2) Generation of a constellation of new prions in vitro leading to the emergence of novel prion diseases.
    		 15:20-15:40 (OC10.2) Single localized treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease.
    15:40-16:00 (OC8.3) Persistent propagation of mouse-adapted vCJD and Fukuoka-1 agents in murine stromal cell cultures of bone marrow and spleen origin.
    		15:40-16:00 (OC10.3) Therapeutic benefit from gene therapy in the curative treatment of prion diseases
    16:00-16:15 (OC8.4) THE SAME PRIMARY STRUCTURE OF THE PRION PROTEIN YIELDS TWO DISTINCT SELF-PROPAGATING STATES
    		 16:00-16:20 (OC10.4) Antibodies directed against the prion protein receptor LRP/LR provide alternative tools in prion diseases
    16:15-16:30 (OC8.5) In vitro studies of the transmission barrier
    		 16:20-16:40 (OC10.5) Italian trial with doxycycline in Creutzfeldt-Jakob disease: background, study protocol and update on study population
    16:30-16:45 (OC8.6) Scrapie production occurs in the the perinuclear recycling compartment
    		16:40-17:00 (OC10.6) High Throughput Screening to Identify Therapeutics for Human Prion Disease
    16:45-17:00 (OC8.7) De novo formation of purified infectious prions from minimal components
    		  
    20:00 Gala Dinner

    Friday 10 October 2008

    Horario Auditorium
    9:00-09:30 (S5) Plenary conference:
    "Reflections on a half-century in the field of Transmissible Spongiform Encephalopathy"
    Dr. Paul Brown
    9:35-10:30 Poster Prizes Presentation
    10:30-11:00 CJD International Support Alliance Session. Working together globally to support those affected by prion disease.
    Sponsored by: Alliance BioSecure

    Suzanne Solvyns. Co-chair of the CJD International Support Alliance. Director – CJD Support Group Network, Australia
    David Ralston. Chair – CJD Support Group Network, Australia
    Gillian Turner. National Coordinator – CJD Support Network, UK
    Roberto Borgis. President - Associazone Italiana Encelfalopatia da Prioni A.I.En.P – Italy
    Raffaella Robello. Vice President - Associazone Italiana Encelfalopatia da Prioni A.I.En.P – Italy
    11:00-11:30 Coffee break
    11:30-13:00 (11) Session 11. Hot topics
    Chair: Jean-Philippe Deslys, Isidre Ferrer
    11:30-11:50 (OC11.1) X-ray fiber diffraction reveals major structural differences between brain-derived prions and recombinant prion protein amyloid
    11:50-12:10 (OC11.2) Exposure to small ruminants TSE agents: a threat for animal and human health?
    12:10-12:30 (OC11.3) PMCA in the bank vole
    12:30-12:50 (OC11.4) Genome-wide association study identifies genetic risk factors for variant Creutzfeldt-Jakob disease
    12:50-13:00 (OC11.5) Prion Protein-deficient Cattle are Resistant to Prion Disease
    13:00-13:30 Closing Ceremony