The Jeffrey A. Smith Memorial Research Grant

Contributed by: The Jeffrey and Mary Smith Family Foundation; Zoë Smith Jaye and Jenny Smith Unruh; and Mary Smith

Established in 2015 

Research Grants Supported

Wen-Quan Zou, MD, PhD

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

Martine Bruley Rosset, PhD

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

Joaquín Castilla, PhD

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

Wen-Quan Zou, MD, PhD

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

Gianfranco Puoti, MD, PhD

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

Maurizio Pocchiari, MD

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

Sylvain Lehmann, MD, PhD

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

Qingzhong Kong, PhD

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

Yury O. Chernoff, PhD

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

Joaquín Castilla, PhD

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

Emiliano Biasini, PhD

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

Wen-Quan Zou, MD, PhD

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

Joel Watts, PhD

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

Paula Saa, PhD

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

Christina Orru, PhD

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

Wen-Quan Zou, MD, PhD

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

Christina Sigurdson, DVM, PhD

Grant Title:
Mechanisms of Selective Neuronal Vulnerability in Human Prion Disease

Location:
University of California, San Diego

Grant Year:
2015

Julie Moreno, PhD

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

Jean-Philippe Deslys, MD

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

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

Valerie Sim, MD, FRCPC

Grant Title:
Combination therapies for human prion disease

Location:
Department of Medicine, University of Alberta

Grant Year:
2016

Zuzana Krejciova, PhD

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

Roberto Chiesa, PhD

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

Byron Caughey, PhD

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

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

Wen-Quan Zou, MD, PhD

Grant Title:
Assessing Prions in Sporadic CJD Patients

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

Grant Year:
2017

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

Charles E. Mays, PhD

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

Stéphane Haik, MD, PhD

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

Rodrigo Morales, PhD

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

Sylvain Lehmann, PhD

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

Allison Kraus, PhD

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

Location:
Rocky Mountain Laboratories, Montana

Grant Year:
2018

David A. Harris, MD. PhD

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

Location:
Department of Biochemistry, Boston University School of Medicine

Grant Year:
2018

Leonardo Cortez, PhD

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

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

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

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

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