The Dr. Luke Baxley GSS Memorial Research Grant
Contributed by: Kathy Baxley
Contributed by: The Jeffrey and Mary Smith Family Foundation; Zoë Smith Jaye and Jenny Smith Unruh; and Mary Smith
Established in 2015
Contributed by: Kathy Baxley
Contributed by: Jana Able

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
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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Grant Title:
Mechanisms of Selective Neuronal Vulnerability in Human Prion Disease
Location:
University of California, San Diego
Grant Year:
2015

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

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

Grant Title:
Pharmacological Chaperones for the Cellular Prion Protein
Location:
Center for Integrative Biology (CIBIO) at the University of Trento, Italy
Grant Year:
2015

Grant Title:
Combination therapies for human prion disease
Location:
Department of Medicine, University of Alberta
Grant Year:
2016

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

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

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

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

Grant Title:
Assessing Prions in Sporadic CJD Patients
Location:
National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio
Grant Year:
2017

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

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

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

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

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

Grant Title:
“Investigation of Prion Inactivation by Reactive Oxygen Species in Vivo”
Location:
Rocky Mountain Laboratories, Montana
Grant Year:
2018

Grant Title:
“Highly Synergistic Combination Therapy for Prion Diseases”
Location:
Department of Biochemistry, Boston University School of Medicine
Grant Year:
2018

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

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

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

Grant Title:
Modeling Creutzfeldt-Jakob Disease Using Human iPSC-derived Neurons
Location:
Center for Regenerative Medicine, Boston University School of Medicine
Grant Year: 2019

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

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

Grant Title:
Cerebral neuronal dysfunction associated with human prion diseases
Location:
National Institute of Allergy and Infectious Diseases, NIH
Grant Year: 2019

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

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

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

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,

December 17, 1955 – Feb. 3, 2006

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

August 1, 1949 – April 8, 2008

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