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Research Grant Recipients

2024

Joaquín Castilla, PhD

Grant Title:
Detection of residual prions from decontaminated medical and laboratory surfaces

Exploring the efficacy of dominant negative protein-based gene therapy for different prion diseases: in vivo proof-of-concept study
Location:
Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain

Grant Year: 2024

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Arielle Hay, Ph.D.

Grant Title:
Generating cerebral organoids from donors with sporadic Creutzfeldt-Jakob Disease

Location:
Postdoctoral Research Fellow at Rocky Mountain Laboratories, National Institutes of Health, Hamilton, Montana

Grant Year: 2024

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Dr. Surabhi Mehra

Grant Title:
Understanding the molecular mechanism of spontaneous prion emergence in knock-in mouse models

Location:
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto

Grant Year: 2024

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Debbie McKenzie, PhD

Grant Title:
Modulation of Toll-Like Receptor Signaling: Unveiling Neuroinflammation and Therapeutic Strategies in Creutzfeldt-Jakob Disease

Location:
University of Alberta

Grant Year: 2024

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Gerold Schmitt-Ulms, PhD

Grant Title:
Bridging the pre-clinical gap for a small brain-penetrant molecule that reduces PrPC levels

Location:
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto

Grant Year: 2024

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Dr. Matthias Schmitz

Grant Title:
Validation of a non-invasive diagnostic test for prion diseases using tear fluid

Location:
Dementia Research Unit, Dept. of Neurology in Göttingen, National Reference Center for TSE and German Center for Neuro-degenerative diseases, Germany

Grant Year: 2024

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2023

Ignazio Cali, PhD

Grant Title:
Investigating prion protein seeding activity in Alzheimer’s disease and chronic traumatic encephalopathy

Location:
Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH

Grant Year: 2023

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Dr. Thomas Cunningham and Elizabeth Hill

Grant Title:
Generation of Humanised STX6 Overexpression Mice to Study Prion Disease Genetic Risk and as a Model for Therapeutic Intervention

Location:
MRC Prion Unit at UCL, Institute of Prion Diseases, London

Grant Year: 2023

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Victoria Lawson, PhD

Grant Title:
Risk of cancer from reduced expression of PrPC-implications for prion disease treatment”

Location:
Department of Microbiology and Immunology, Peter Doherty Institute for Infection and Immunity, The University of Melbourne, Victoria, Australia

Grant Year: 2023

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Qiuye Li, Ph.D.

Grant Title:
“High-resolution structure determination of MM1 and MM2 sCJD prions”

Location:
Department of Physiology and Biophysics, Case Western Reserve University School of Medicine, Cleveland, OH

Grant Year: 2023

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Robert C.C. Mercer, Ph.D.

Grant Title:
The identification of PrPSc interaction partners and the characterization of their role in prion infection and/or propagation

Location:
Instructor, Department of Biochemistry, Boston University School of Medicine, Boston, MA

Grant Year: 2023

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Satish Nemani, PhD and Valerie L Sim, MD, FRCPC

Grant Title:
Contribution of oligomeric prion populations to phenotypic heterogeneity in variably protease-sensitive prionopathy (VPSPr) versus silent prions

Location:
Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada

Grant Year: 2023

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Dr. Sarah Vascellari

Grant Title:
Development of a blood and urine prion diagnostic test for human prion diseases using the Real Time QuIC assays

Location:
Department of Biomedical Sciences, University of Cagliari (UniCa), Cagliari, Italy

Grant Year: 2023

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2022

Hermann Clemens Altmeppen, PhD

Grant Title:
Enzymatic PrP shedding in humans: Biomarker potential and therapeutic target

Location:
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

Grant Year: 2022

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Maria Letizia Barreca, PhD

Grant Title:
Blocking the Neurotoxic Activities of Mutant Prion Proteins by a Novel Class of Therapeutic Agents

Location:
Department of Pharmaceutical Sciences (DPS), University of Perugia-Italy

Grant Year: 2022

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Marcelo Barria, PhD & Alison Green, PhD

Grant Title:
Assessing the replication properties of prion protein aggregates found in archived formalin-fixed, paraffin embedded appendix tissue collected from the asymptomatic population by the highly sensitive in vitro prion amplification assay.

Location:
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK

Grant Year: 2022

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Joaquín Castilla, PhD

Grant Title: Development and characterization of the fastest animal model able to propagate GSS prions faithfully

Location:
Center for Cooperative Research in Biosciences (CIC bioGUNE), Basque Research and Technology Alliance (BRTA), Derio, Spain

Grant Year: 2022

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Cathryn Haigh, PhD

Grant Title:
“Regional tropism of sporadic CJD subtypes in human brain organoids”

Location:
Laboratory of Neurological Infections and Immunity (LNII), National Institute of Allergy and Infectious Diseases (NIAID), Division of Intramural Research, Rocky Mountain Laboratories, National Institutes of Health, Hamilton, MT

Grant Year: 2022

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Rodrigo Morales, PhD

Grant Title:
Development of a high throughput system for screening anti-prion molecules

Location:
University of Texas, Houston, TX

Grant Year: 2022

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Jaime Noguez

Grant Title:
Improving the sensitivity of CSF RT-QuIC in humans by examining cases with indeterminate results

Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, University Hospitals Cleveland Medical Center, Cleveland, OH

Grant Year: 2022

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Shashirekha Shetty, PhD

Grant Title:
Examining copy number variants as a possible phenotype modifier in prion disease

Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, University Hospitals, Cleveland, OH

Grant Year: 2022

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2021

Alberto Bizzi, MD

Grant Title:
Early diagnosis of CJD from in vivo MRI using the trajectories of prion lesion propagation

Location:
Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

Grant Year: 2021

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Stephanie Booth, PhD

Grant Title:
Detection and characterization of rare strains of sporadic Creutzfeldt-Jakob Disease using a suite of novel biological and biochemical tools

Location:
Public Health Agency of Canada and University of Manitoba.

Grant Year: 2021

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Jason Thomas Duskey and Barbara Ruozi

Grant Title:
Optimization of nanoparticle-mediated brain delivery of a tetracationic porphyrin with potent anti-prion activities

Location:
University of Modena and Reggio Emilia, Modena, Italy

Grant Year: 2021

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Franc Llorens, PhD and Inga Zerr, MD

Grant Title:
Implementation of a blood-based biomarker test for sporadic Creutzfeldt-Jakob in clinical practice”

Location:
The Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Spain and German Center for Neurodegenerative Diseases

Grant Year:
2021

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Simon Mead, PhD

Grant Title:
Genome-wide association study (GWAS) of age at clinical onset in inherited prion disease (genetic CJD)

Location:
University College London (UCL) Institute of Prion Diseases

Grant Year: 2021

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Valerie Sim, MD, FRCPC

Grant Title:
Strain-specific pathology and spread in prion organotypic slice culture assay infected with different strains of Creutzfeldt-Jakob Disease

Location:
Centre for Prions and Protein Folding Diseases, University of Alberta, Canada

Grant Year: 2021

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Holger Wille, PhD

Grant Title:
Evaluating prion vaccines in transgenic mouse models of familial prion diseases

Location:
University of Alberta, Edmonton, Alberta, Canada

Grant Year: 2021

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2020

Natalia do Carmo Ferreira de Araujo

Grant Title:
Evaluation of new anti-prion drugs: from drug design to therapeutic efficacy against human prions in vitro, ex-vivo and in vivo

Location:
Rocky Mountain Laboratories, NIH/NIAID

Grant Year: 2020

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Qingzhong Kong, PhD

Grant Title:
The RAP approach for effective prevention and treatment of prion diseases

Location:
Case Western Reserve University

Grant Year:
2020

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Rodrigo Morales, PhD

Grant Title:
Exploring the zoonotic potential of porcine-derived materials from animals exposed to infectious prions

Location:
Department of Neurology, The University of Texas Health Science Center at Houston

Grant Year: 2020

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Anna Poleggi, PhD

Grant Title:
Investigating the influence of CYP4X1 gene on age at onset of CJD as potential preventive therapeutic approach

Location:
Istituto Superiore di Sanità, Dept. of Neuroscience, Rome

Grant Year: 2020

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Sonia Vallabh

Grant Title:
Assessing efficacy of zinc-finger repressors of prion protein as a therapeutic for prion disease

Location:
Broad Institute of MIT and Harvard

Grant Year: 2020

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Fei Wang, PhD

Grant Title:
Generating Recombinant Human Prions in Large Scale for Structural Studies

Location:
The Mitchell Center for Alzheimer’s Disease and Related Brain Disorders; The University of Texas Health Science Center at Houston

Grant Year: 2020

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Candace Mathiason, PhD

Grant Title:
“Use of Blood-borne Prion Amplification Assays for the Detection of Human Prion Diseases and Biomarker Assessment”

Location:
Prion Research Center, Colorado State University

Grant Year:
2020

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2019

Steven E. Arnold, MD

Grant Title: Assessing long-term stability of cerebrospinal fluid PrP levels in genetic prion disease mutation carriers

Location: Professor of Neurology, Harvard Medical School; and Translational Neurology Head of the Interdisciplinary Brain Center, Massachusetts General Hospital

Grant Year: 2019

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Roberto Chiesa, PhD

Grant Title:
“Nanoparticle-mediated brain delivery of a tetracationic porphyrin with potent anti-prion activities”

Location:
Laboratory of Prion Neurobiology, Department of Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri, Milano, Italy

Grant Year: 2019

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Dr. Graham S. Jackson

Grant Title: Development of a quantitative, real-time Protein-Misfolding by Cyclic Amplification (PMCA) reaction
Location: Medical Research Council (MRC) Prion Unit at University College London
Grant Year: 2019

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Jiyan Ma, PhD

Grant Title:
Determining the therapeutic potential of anti-PrP nanobody

Location: Professor, Center for Neurodegenerative Science, Van Andel Institute, Grand Rapids, MI

Grant Year: 2019

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Gustavo Mostoslavsky, MD, PhD

Grant Title:
Modeling Creutzfeldt-Jakob Disease Using Human iPSC-derived Neurons

Location:
Center for Regenerative Medicine, Boston University School of Medicine

Grant Year: 2019

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Dr. Emmanuelle Vire

Grant Title:
Investigating the relevance of blood DNA methylation signals for disease management in CJD

Location:
Medical Research Council (MRC) Prion Unit at University College London

Grant Year: 2019

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2018

Dr. Hermann Clemens Altmeppen

Grant Title:
“Investigating the Potential of the Neuroprotective N1 Fragment of the Prion Protein as a New Treatment Against Prion Diseases”

Location:
Institute of Neuropathology, University Medical Center – Eppendorf, Hamburg, Germany

Grant Year:
2018

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Leonardo Cortez, Ph.D.

Grant Title:
“Isolation and Strain-Specific Characterization of Pathogenic CJD Prion Particles”

Location:
Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada

Grant Year:
2018

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David A. Harris, M.D., Ph.D.

Grant Title:
“Highly Synergistic Combination Therapy for Prion Diseases”

Location:
Department of Biochemistry, Boston University School of Medicine

Grant Year:
2018

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Allison Kraus, Ph.D.

Grant Title:
“Investigation of Prion Inactivation by Reactive Oxygen Species in Vivo”

Location:
Rocky Mountain Laboratories, Montana

Grant Year:
2018

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Sylvain Lehmann, Ph.D.

Grant Title:
“Innovative Human 3D Neural Network Model for the Efficient Propagation of Human Prions”

Location:
Institute for Regenerative Medicine and Biotherapy, University of Montpellier, France

Grant Year:
2018

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Rodrigo Morales, Ph.D.

Grant Title:
“Systematic Evaluation of the Zoonotic Potential of Different CWD Isolates”

Location:
Mitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas-Houston Medical School

Grant Year:
2018

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2017

Stéphane Haik, M.D., Ph.D.

Grant Title:
Worms: A novel genetic model for therapeutic research in prion disease

Location:
Alzheimer’s and Prion diseases Lab at the Brain & Spine Institute (ICM) of the Salpêtrière Hospital, Sorbonne Université, Paris, France

Grant Year:
2017

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Charles E. Mays, Ph.D.

Grant Title:
Design and Development of Anti-Prions as a Novel Therapeutic Strategy for Prion Disease

Location:
Mitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas-Houston Medical School

Grant Year:
2017

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Gianluigi Zanusso, MD, PhD

Grant Title:
RT-QuIC assay on olfactory brushings in asymptomatic carriers of E200K mutation: An explorative study for establishing when a preventive therapy should be started

Location:
University of Verona, Italy

Grant Year:
2017

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Wen-Quan Zou, M.D., Ph.D.

Grant Title:
Assessing Prions in Sporadic CJD Patients

Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio

Grant Year:
2017

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2016

Dr. Hermann Clemens Altmeppen

Grant Title:
Targeting the endogenous proteolytic processing of PrPc as a therapeutic strategy against prion diseases

Location:
Institute of Neuropathology, University Medical Center Eppendorf

Grant Year:
2016

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Byron Caughey, Ph.D.

Grant Title:
Antisense Oligonucleotides to delay or prevent onset of prion disease in mice

Location:
National Institute of Allergy and Infectious Diseases, National Institute of Health

Grant Year:
2016

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Roberto Chiesa, Ph.D.

Grant Title:
A new anti-prion compound that reduces PrPc levels and inhibits PrPsc replication. [Awaiting permission to release from Dr. Chiesa.]

Location:
Istituto di Ricerche Farmacologiche Mario Negri, Milano, Italy

Grant Year: 2016

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Zuzana Krejciova, Ph.D.

Grant Title:
Human stem cell derived neural models for sCJD prion propagation and drug discovery

Location:
Institute for Neurodegenerative Diseases, University of California, San Francisco

Grant Year:
2016

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2015

Emiliano Biasini, PhD

Grant Title:
Pharmacological Chaperones for the Cellular Prion Protein

Location:
Center for Integrative Biology (CIBIO) at the University of Trento, Italy

Grant Year:
2015

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Jean-Philippe Deslys, M.D.

Grant Title:
Human Mini-Brains as a New Platform to Identify and Validate Pharmaceutical Compounds for the Treatment of CJD

Location:
CEA/DSV/iMETI/SEPIA (Institute for Emerging Illnesses and Innovative Therapies, Department of Prion and Atypical Infections Research), Fontenay-Aux-Roses, France

Grant Year:
2015

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Julie Moreno, Ph.D.

Grant Title:
The Role of Cellular Chaperones and the Unfolded Protein Response in Gerstmann-Sträussler-Scheinker Syndrome

Location:
Prion Research Center, Colorado State University

Grant Year:
2015

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Wen-Quan Zou, M.D., Ph.D.

Grant Title:
Developing Therapeutics for CJD Using Patient-Specific iPSC-Derived Neurons

Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine Cleveland, Ohio

Grant Year:
2015

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2012

Christina Orru, Ph.D.

Grant Title:
Development of an Anti-Mortem and Pre-Symptomatic Diagnostic Test for vCJD and Genetic Human Prion Diseases Using Real Time QuIC

Location:
Department of Biomedical Sciences, University of Cagliari, Cittadella Universitaria, Monserrato (CA) — Italy

Grant Year:
2012

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Paula Saa, Ph.D.

Grant Title:
In Vitro and In Vivo Modeling of Gerstmann-Sträussler-Scheinker Syndrome (GSS) With Spontaneously Generated Human Recombinant PrPres Harboring the P102L Substitution (P102L-rPrPres)

Location:
American Red Cross, Scientific Affairs, Rockville, Maryland

Grant Year:
2012

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Joel Watts, Ph.D.

Grant Title:
Propagation of CJD Prions in Cultured Cells Expressing Bank Vole PrP

Location:
Institute for Neurodegenerative Diseases, University of California, San Francisco

Grant Year:
2012

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Wen-Quan Zou, M.D., Ph.D.

Grant Title:
Probing CJD and GSS by Generating Patient-Derived Induced Pluripotent Stem Cells (iPSCs)

Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University School of Medicine, Cleveland, Ohio

Grant Year:
2012

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2011

Emiliano Biasini, Ph.D.

Grant Title:
Targeting the Functional Activity of PrPc as a Novel Strategy for Drug Discovery in Prion Diseases

Location:
Harris Laboratory, Boston University, Boston, Massachusetts

Grant Year:
2011

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Joaquín Castilla, Ph.D.

Grant Title:
Developing a Prion Susceptible Human Neuronal Cell Line Based on Reprogramming Fibroblasts from CJD Affected Patients

Location:
Prion Lab, Proteomic Unit, CIC bioGune, Bizkaia, Spain

Grant Year:
2011

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Yury O. Chernoff, Ph.D.

Grant Title:
Yeast Model for Studying Heritable Mammalian Prion Diseases in Mammals

Location:
School of Biology, Georgia Institute of Technology, Atlanta, Georgia

Grant Year:
2011

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2009

Qingzhong Kong, Ph.D.

Grant Title:
Etiology of Sporadic CJD: Somatic Mutations of the Human Prion Protein Gene in Brain Cells

Location:
The National Prion Disease Pathology Surveillance Center, Cleveland, Ohio

Grant Year:
2009

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Sylvain Lehmann, M.D., Ph.D.

Grant Title:
Adult Neurogenesis in Prion Diseases: A New Lead for Therapeutics?

Location:
Department of Molecular and Cellular Pathologies, Institut de Genetique Humaine, Montpellier, France

Grant Year:
2009

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Maurizio Pocchiari, M.D.

Grant Title:
Pentosan Polysulphate: A Friendly Delivery System for Therapy in Prion Diseases

Location:
Clinical, Diagnosis and Therapy of Neurodegenerative Disorders Unit, Department of Cell Biology and Neurosciences, Instituto Superiore di Sanita, Rome, Italy

Grant Year:
2009

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Gianfranco Puoti, M.D., Ph.D.

Grant Title:
Analyses of the Protein Tau to Help Determine Whether the Newly Discovered Protease-Sensitive Prionopathy is the Sporadic Form of Gerstmann-Sträussler-Scheinker Disease (GSS)

Location:
The National Prion Disease Pathology Surveillance Center, Cleveland, Ohio

Grant Year:
2009

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Wen-Quan Zou, M.D., Ph.D.

Grant Title:
“Assessing long-term stability of The CJD Foundations 2009 Research Grant Renewal: Comparative Study of Prions Associated with Iatrogenic and Sporadic Creutzfeldt-Jakob Disease: An Update

Location:
Department of Pathology, Case Western Reserve University; Associate Director of National Prion Disease Pathology Surveillance Center, Cleveland, Ohio

Grant Year:
2009

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2008

Joaquín Castilla, Ph.D.

Grant Title:
Searching for Custom Dominant Negative PrPs by In Vitro Prion Replication Studies

Location:
Prion Lab, Proteomic Unit, CIC bioGune, Bizkaia, Spain

Grant Year:
2008

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Martine Bruley Rosset, Ph.D.

Grant Title:
Studies of Immune Responses to Prion Protein (PrP) During Prion Infection: Role of Regulatory T cells

Location:
Institut National de la Santé et de la Recherche Médicale, Paris, France

Grant Year:
2008

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2006

Wen-Quan Zou, M.D., Ph.D.

Grant Title:
Comparative Study of Prion Strains Associated with Iatrogenic and Sporadic Creutzfeldt-Jakob Disease

Location:
Case Western Reserve University, Cleveland, Ohio, Recipient of the first CJD Foundation Human Research Grant, Cleveland, Ohio

Grant Year:
2006

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