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

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

Simote Totauhelotu Foliaki, PhD

Grant Title:
Cerebral neuronal dysfunction associated with human prion diseases

Location:
National Institute of Allergy and Infectious Diseases, NIH

Grant Year: 2019

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

Nicholas J. Zazzera Sr.

Back to Quilt There are many ways of honoring someone. Nicholas J. Zazzera Sr. was someone who deserved to be honored. There is a tradition

Barbara Zane

Back to Quilt In loving memory of Barbara Zane, a special sister and friend who died of CJD on 5/10/06. She is sorely missed. “What

Gerard “Jerry” Zajac

Back to Quilt Jerry passed away at his home in Tampa on November 13, 2016.  Jerry was a man who loved people; he listened, talked,

Paulette Wilson

Back to Quilt A loving wife, mother, and Yiayia.  We think about you every day, and we know where you are. We love you.  

Anne Marie Whalen

Back to Quilt In memory of the most beautiful, caring, loving wife, mom, sister and friend. We love and miss you everyday!