2015 CJD Foundation Family Conference Speakers

Byron Caughey, PhD
Keynote Speaker: Progress in Detecting Prions and Diagnosing Prion Diseases
Byron Caughey, PhD, is a biochemist, Senior Investigator and Chief of the TSE/Prion Biochemistry Section of the Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories (RML) in Hamilton, Montana. RML is part of the National Institute of Allergy & Infectious Diseases (NIAID), National Institutes of Health. Dr Caughey has been on the editorial boards of several journals and is currently a senior editor for the Journal of Virology. Moreover, he has served on the scientific advisory boards of agencies that fund TSE/prion disease research such as PrioNet Canada and Alberta Prion Research Institute and reviewed many grants and prion disease research programs on an ad hoc basis.

Dr. Caughey got his Ph.D. in biochemistry from the University of Wisconsin-Madison in 1985. After post-doctoral research in neurochemistry at Duke University, he joined the Rocky Mountain Labs in 1986 to begin studies of the transmissible spongiform encephalopathies (TSEs) or prion diseases.

Dr. Caughey now leads one of the several TSE research labs at Rocky Mountain Labs. Key questions that his lab has addressed include the following: What is the infectious agent of TSE diseases of humans and animals? How is prion protein corrupted (misfolded) and spread in TSE diseases? How can we prevent prion protein misfolding? How can we better diagnose and treat TSE diseases?

Dr. Caughey’s lab and their collaborators have made a number of discoveries, including: 1) initial descriptions of when and where in infected cells that prion protein is converted to the TSE-associated form, 2) the first demonstration of the ability of the TSE-associated prion protein to corrupt its normal counterpart, and the strain dependence of that activity, 3) the identification of the first, and many other, inhibitors of TSE-associated prion protein formation, i.e. potential drugs with anti-TSE activity in animals, 4) the development of ultrasensitive, rapid and practical new tests for prions and TSE diagnosis, 5) the first experimental assessment of the misfolded structure of TSE-associated prion protein and how it varies with TSE strain, 6) an assessment of the most infectious prion protein particles, and 7) the first visualization of the uptake and transport of infectious prion protein particles in live neuronal cells.  This and other work has resulted so far in >180 research papers.

Click here to view Dr. Caughey's 2015 presentation.


Jiri Safar, MD, PhD, Director, National Prion Disease Pathology Surveillance Center, Associate Professor, Department of Pathology, Case Western Reserve University
New Goals and Organizational Structure of the National Prion Disease Pathology Surveillance Center (NPDPSC)

Dr. Jiri G. Safar earned his MD from the School of Medicine at Charles University, Prague, Czech Republic. After his Board Certification in Neurology and PhD training in biochemistry, he received a Research Fellowship in the Laboratory of Central Nervous System Studies at the National Institutes of Health (NIH), Bethesda, Maryland, directed by D.C. Gajdusek (Nobel Prize for Medicine). He continued his research at the NIH as a Senior Scientist and received the NIH Award of Merit for his work on molecular pathology of prion proteins. In 1996 he took the Associate Professor position in the Department of Neurology at the University of California at San Francisco (UCSF) and Senior Scientist position at the Institute for Neurodegenerative Diseases, directed by Stanley B. Prusiner (Nobel Prize for Medicine). In 2008, Dr. Safar established a new prion research lab at the Department of Pathology, Case Western Reserve University (CWRU), Cleveland, Ohio, and in 2015 was appointed the Director of the National Prion Diseases Pathology Surveillance Center at CWRU. He is an Executive Committee Member of the Case Brain Health Collaborative Initiative that is developing a strategy to establish a new Brain Health Center at CWRU to be dedicated to the translational medicine of neurodegenerative diseases.

Dr. Safar has authored 190 publications and holds 27 U.S. and international patents.  He served as a member of the British Spongiform Encephalopathy Advisory Committee (SEAC) in the United Kingdom from 1999 to 2003.  He is currently a member of the Transmissible Spongiform Encephalopathy Advisory Committee (TSEAC) at Food and Drug Administration (FDA), European Community Prion Expert Group, World Health Organization (WHO), and Medical Co-director of Creutzfeldt-Jakob Disease Foundation.  Dr. Safar’s extensive research has led to the discovery of previously unknown forms of prions and prion diseases, a better understanding of the conformation of prion proteins, the unraveling of the molecular mechanisms of prion strains, and the development of new methods of prion detection.

Click here to view Dr. Safar's 2015 presentation.


Emiliano Biasini, PhD
Pharmacological Chaperones for the Cellular Prion Protein
Dr. Biasini is an Assistant Telethon Scientist for the Dulbecco Telethon Institute at the Center for Integrative Biology of the University of Trento (Italy). He also holds a position of Research Fellow of the Italian Ministry of Health at the Mario Negri Institute for Pharmacological Research in Milan.

Dr. Biasini has more than 15 years of experience in the field of prion diseases. He received his Ph.D. in 2005 from the University of La Sapienza in Rome, and a specialization in Pharmacological Research from the Mario Negri Institute in Milan. From 2005, he worked as a postdoctoral fellow in the laboratory of Dr. David Harris at Washington University in St. Louis, and in 2010 was appointed Research Instructor at Boston University.

The main objective of his laboratory is to develop novel therapeutic strategies for prion disorders and Alzheimer’s disease.

Click here to view Dr. Biasini's 2015 presentation.


Jean-Philippe Deslys, MD, PhD
Human Mini-brains as a New Platform to Identify and Validate Pharmaceutical Compounds for the Treatment of CJD
Dr. Deslys co-authored more than one hundred publications in international scientific journals on the main aspects of applied prion research (diagnostic, decontamination techniques, risk assessment, and therapeutic approaches in different experimental models) and on underlying pathological mechanisms. He studied the genetic of the first cases of iatrogenic CJD in France. His work has led to several patents including the BSE (Bovine Spongiform Encephalopathy) diagnostic test most widely used worldwide. He also wrote a book on mad cow disease which can be downloaded here for free (http://www.neuroprion.org/pdf_docs/documentation/madcow_deslys.pdf). His research group is Associate Laboratory to National Reference Laboratory for CJD in France and has high security level microbiological installations (NeuroPrion research platform) with different experimental models (mouse, hamster, monkey). For several years, his interest has extended to the connections between PrP and Alzheimer and the prion mechanisms underlying neurodegenerative diseases. His group develops new tools to study these different phenomena in a perspective of shared expertise (project 3D NeuroSecure) and has successfully implemented the model of IPSc and human minibrains which offers exiting new insights for future therapeutics.

Click here to view Dr. Deslys' 2015 presentation.


Amanda Kalinsky, RN BSN
Where Do We Go From Here?

Amanda Kalinsky graduated from the College of Charleston in 2006 with a B.A. in Biology. She then received her B.S. in Nursing in 2008 from the Medical University of South Carolina. She works as a rehabilitation nurse specializing in spinal cord injuries and traumatic brain injuries at Roper Hospital in Charleston, SC. Amanda is a mother of three; her children include twins Ava and Cole (4 years old) and Tatum (2 years old). She also serves on the CJD Foundation’s Board of Directors as the Reproductive Options Consultant.

In 2004, Amanda's father, Luke D. Baxley, MD, was diagnosed with Gerstmann-Sträussler-Scheinker disease. He passed away in April 2010 of complications resulting from the disease. In April 2009, Amanda tested positive for the F198S mutation, one of the genetic mutations responsible for causing Gerstmann-Straussler-Scheinker disease and the same one her father carried. In order to not pass this genetic mutation onto her children, Amanda chose to undergo Invitro Fertilization with Preimplantation Genetic Diagnosis through the Reproductive Genetics Institute in Chicago, IL. Since going through the process and having healthy twins in January 2011, Amanda has become passionate about educating others with or at risk of having an inheritable prion disease about IVF with PGD as a reproductive option.

Click here to view Amanda's 2015 presentation.


Bradley Kalinsky, MD
After IVF with PGD

Bradley Kalinsky received his medical degree from the Medical University of South Carolina. He recently completed a Nephrology fellowship at Vanderbilt Medical Center and, in August, will start a new job in Charleston, SC. Bradley married Amanda Kalinsky in January of 2010 and is now the proud father of 4 year old twins, Ava and Cole, and 2 year old Tatum.

The couple currently reside in Mount Pleasant, SC with their three children and dog Sadie.

Click here to view Dr. Kalinsky's 2015 presentation.


Kevin Keough, PhD, FCAHS
Alberta Prion Research Institute (APRI) Report

Dr. Kevin Keough is currently the Executive Director of the Alberta Prion Research Institute.  Prior to joining the Alberta Prion Research Institute, Dr. Keough held various senior positions within the research sector, including: President and CEO of the Alberta Heritage Foundation for Medical Research ; the first Chief Scientist at Health Canada in Ottawa; and, the first Vice-President of Research and International Relations at Memorial University of Newfoundland.  He is also co-founder, President and Chief Scientific Officer of NovaLipids Inc., the Principal of Kevin Keough Consulting Inc., and a Fellow of the Canadian Academy of Health Sciences.  He has had an active career in research for nearly fifty years.

Dr. Keough has been a member of the Boards of Directors of a large number of agencies and not-for-profit corporations, and is currently on the Boards of the Alberta Centre for Advanced MNT Products and the Science Media Centre of Canada.  He has served as a consultant to national and international research organizations, the pharmaceutical industry and to various levels of government.  He continutes to participate in research-related activities and is frequently consulted on issues of science and innovation policy and management by academia, government and the private sector.

Click here to view Dr. Keough's 2015 presentation.


Ryan A. Maddox, PhD
The Centers for Disease Control and Prevention Report: Prion Disease Activities at the CDC

Ryan Maddox is an epidemiologist with the National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention (CDC). In this role, Dr. Maddox coordinates nationwide surveillance of human prion diseases, including Creutzfeldt-Jakob disease. He also conducts national surveillance of Kawasaki syndrome.

Dr. Maddox received his Bachelor of Science from Auburn University in 1998. In 2000, he earned his Master of Public Health in epidemiology from Emory University’s Rollins School of Public Health in Atlanta, Georgia, and he earned his PhD from the same institution in 2012. Dr. Maddox is the author or co-author of many publications covering various aspects of prion diseases in the United States.

Click here to view Dr. Maddox's 2015 presentation.


Julie Moreno, PhD
The Role of Cellular Chaperones and the Unfolded Protein Response in Gerstmann-Sträussler-Scheinker Syndrome

Julie Moreno, PhD is a Post Doctoral Fellow at Colorado State University, Prion Research Center, Fort Collins, Colorado in Dr. Glenn Telling’s group.

Dr. Moreno graduated with her PhD in Cell and Molecular Biology in 2009 from Colorado State University in Dr. Ron Tjalkens laboratory. As a PhD student, she identified a role for the NF-B pathway in astrocyte induced neuroinflammation in a mouse and cellular model of manganese neurotoxicity that was found to be dependent on age and gender. Following her PhD, she joined the laboratory of Professor Giovanna Mallucci as a postdoctoral fellow at the Medical Research Council Toxicology Unit in Leicester, England.  There she found that the accumulation of misfolded prion protein (PrP) leads to activation of the unfolded proteins response (UPR) and neurodegeneration in prion disease. These studies were highly significant in the field and resulted in two publications in Nature and Science Translational Medicine, recommended by F1000Prime and received world-wide media attention. Currently, she is a post-doctoral fellow in Dr. Glenn Telling’s laboratory at Colorado State University in the Prion Research Center. Her current research is to investigate cellular factors and mechanisms important in prion propagation and disease, using various human and animal models.

Click here to view Dr. Moreno's 2015 presentation.


Christina Sigurdson, DVM, PhD
Mechanisms of Selective Cell Vulnerability in Human Prion Disease
Dr. Christina Sigurdson received her D.V.M. at the University of California, Davis in 1994 and a Ph.D. funded by an NIH fellowship at Colorado State University (CSU) in 2001. In 2002, she completed an anatomic pathology residency at CSU and became a Diplomat of the American College of Veterinary Pathologists.  She then pursued postdoctoral studies of prion diseases at the University of Zürich, Switzerland with an award from the U.S. Department of Defense. In 2008, Dr. Sigurdson joined the faculty at the University of California at San Diego and at Davis. Dr. Sigurdson has served as a scientific advisor in European Union committees as well as participated in international scientific meetings on protein misfolding disorders. She has been working in the prion field for 17 years.

Click here to view Dr. Sigurdson's 2015 presentation.


Wenquan Zou, MD, PhD
Developing Therapeutics for CJD Using Patient-Specific iPSC-Derived Neurons

Dr. Zou is the Associate Professor from the Departments of Pathology and Neurology, and Associate Director of National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH.

Dr. Zou received his medical degree from Jiangxi Medical College and his Ph.D. from Shanghai Medical University. He has practiced Internal Medicine and Nephrology in China. His post-doctoral work in neurodegenerative diseases, with a concentration in prion diseases, was done at the Institute of Pathology, Case Western Reserve University and at the Centre for Research in Neurodegenerative Diseases, University of Toronto.

Dr. Zou’s research focus is in the area of prion disease. Currently, the Zou laboratory is developing patient-specific authentic cell models for studying human prion diseases and for developing therapeutic strategy for prion disease.

Click here to view Dr. Zou's 2015 presentation.