2016 CJD Foundation Family Conference Speakers

John Collinge, MD, CBE, FRS
Keynote Speaker
John Collinge is Professor of Neurology and Head of the Department of Neurodegenerative Disease at the UCL Institute of Neurology and Director of the UK Medical Research Council (MRC) Prion Unit in London, England. He also directs the NHS National Prion Clinic at the adjacent National Hospital for Neurology and Neurosurgery. Prof. Collinge trained in medicine at the University of Bristol and in neurology at St. Mary’s Hospital and the National Hospital for Neurology and Neurosurgery in London.

His laboratory demonstrated in 1996 that the new human prion disease, variant CJD, was caused by the same prion strain as that causing BSE in cattle and has been responsible for a number of key advances in the field. Prof. Collinge has served on numerous Government advisory committees on prion disease at a national, European Union and international level.

He is a Fellow of the Royal College of Physicians, a Fellow of the Royal College of Pathologists, a Founder Fellow of the UK Academy of Medical Sciences and a Fellow of the Royal Society. He was awarded a CBE for services to medical research by HM the Queen. He was recently elected an Honorary Fellow of the American Neurological Association.


Jiri Safar, MD
NPDPSC Overview and Panel on Diagnostics: Recent Advances
Dr. 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 Disease 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.


Joel Watts, Ph.D.
The Role of Animal Models in Identifying and Testing Treatments
Following an undergraduate degree in biochemistry at the University of Western Ontario, Dr. Joel Watts joined the laboratory of Dr. David Westaway at the University of Toronto where he received his Ph.D. in Laboratory Medicine and Pathobiology.

Dr. Watts recently completed his postdoctoral studies in the lab of Dr. Stanley Prusiner at the University of California, San Francisco. In February 2014, Dr. Watts was appointed as an Assistant Professor within the Tanz Centre for Research in Neurodegenerative Diseases at the University of Toronto.

The research interests of Dr. Watts include the prion diseases and related human neurodegenerative disorders such as Alzheimer’s disease and Parkinson’s disease. His research focuses on developing transgenic mouse and cellular models of neurodegenerative diseases that can be used to study the biology of these illnesses and as tools for developing novel therapeutics.

Click here to view Dr. Watts' 2016 presentation.


Zuzana Krejciova, Ph.D.
Human Stem Cell Derived Neural Models for sCJD Prion Propagation and Drug Discovery

Dr. Zuzana Krejciova is a postdoctoral researcher at the Prusiner Laboratory at the Institute for Neurodegenerative Diseases, University of California, San Francisco, where her work focuses on developing human cell culture models of prion infection, using stem cells differentiated toward brain cells.

Dr. Krejciova received her Ph.D. from the National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences at the University of Edinburgh, United Kingdom. In her thesis work, she studied the potential susceptibility of human tonsil cells and stem cells to prion propagation. She received her bachelor’s degree in Biology and master’s in Molecular Biology from Comenius University, Bratislava, Slovakia.

Click here to view Dr. Krejciova's 2016 presentation.


Hermann Clemens Altmeppen
Targeting the Endogenous Proteolytic Processing of PrPc as a Therapeutic Strategy Against Prion Diseases

Hermann Clemens Altmeppen studied Human Biology (Biomedical Sciences) with a strong focus on cell biology, biochemistry and infectious diseases at Philipps University in Marburg, Germany.

After experiencing various research internships in these subjects in laboratories in Marburg, Vienna (Austria) and San Francisco (USA), he successfully accomplished his Diploma thesis in 2009 with a study on the proteolytic processing of viral fusion proteins in the Institute of Virology, Marburg. He then moved to Hamburg to join the Institute of Neuropathology directed by Prof. Dr. Markus Glatzel for his doctoral thesis project on the proteolytic cleavage of the prion protein. He obtained his doctoral degree in 2013. Since that time, he has further developed this subject as a postdoctoral fellow.

Since 2014 he has participated in the collaborative research center on "Proteolysis as a regulatory event in pathophysiology" (CRC 877) in Kiel and Hamburg. His current research focuses on how proteolytic processing events on key proteins impact on neurodegenerative diseases, such as rare and transmissible prion diseases or Alzheimer’s disease, the most frequent cause of dementia, with the overall goal of devising novel treatment options against these fatal conditions.

Click here to view Dr. Altmeppen's 2016 presentation.


Byron Caughey, PhD
Antisense Oligonucleotides to Delay or Prevent Onset of Prion Disease in Mice

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 2016 presentation.


Roberto Chiesa, Ph.D.
A New Anti-Prion Compound that Reduces PrPc Levels and Inhibits PrPsc Replication

Roberto Chiesa graduated in Biology (majoring in Genetics) at the University of Pavia, Italy, in 1991, and obtained a Ph.D. in Pharmacology at the Mario Negri Institute for Pharmacological Research of Milan in 1994.

From 1996 through 2000 he was Research Associate at the Department of Cell Biology and Physiology of Washington University in St. Louis, Mo. In 2001 Dr. Chiesa moved back to the Mario Negri Institute where he held a Telethon Scientist position (Dulbecco Telethon Institute, Telethon Foundation) until December 2013.

Since 2009, he has been the head of the Laboratory of Prion Neurobiology. His research activity is mainly focused on the molecular mechanisms of neurodegeneration in genetic prion diseases. Dr. Chiesa received the James L. O’Leary Prize (1998) and Bruno Ceccarelli Prize (2000) for research in neuroscience. He is a member of the editorial boards of PloS ONE and Biochemical Journal.

Awaiting permission to release from Dr. Chiesa.


Valerie Sim
Combination Therapies for Human Prion Disease

Dr. Valerie Sim self-identifies as a scientist who practices medicine to support her music habit. By day she is an associate professor, neurologist and clinician scientist at the Centre for Prions and Protein Folding Diseases at the University of Alberta in Edmonton, Alberta, Canada. By night, she is a violinist and fiddler.

After earning her undergraduate and medical degrees at the University of Calgary and her neurology residency at University of Ottawa, Dr. Sim completed a post-doctoral fellowship in prion disease research at Rocky Mountain Laboratories, NIH, Montana, under the supervision of Dr. Byron Caughey. She joined the University of Alberta Division of Neurology in 2009 and was promoted to associate professor in July 2016.

In her research lab, Dr. Sim grows prion-infected brain slices in a dish in order to ask: 1) how a prion’s size and shape can influence patterns of disease and risks of transmission; and 2) how targeting multiple steps along the disease pathway might produce more effective treatments. From protein biochemistry to animal treatment experiments, her research publications have received international media attention. She also promotes science communication and regularly presents the science of prion disease to diverse communities across Alberta, Canada.

Clinically, Dr. Sim is a consultant for rapidly progressive dementia cases locally and throughout Canada. She is also the medical director of CJD Foundation Canada, a recently founded family support resource.

From a musical perspective, Dr., Sim never says no to an opportunity to entertain, and she believes that music itself is inherently therapeutic, even if not anti-prion per se. Judge for yourself …

Click here to view Dr. Sim's 2016 presentation.