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Jiri G. Safar, MD

Director, National Prion Disease Pathology Surveillance Center
Associate Professor

Jiri G. Safar is the Director of the National Prion Disease Pathology Surveillance Center, and Associate Professor in the Departments of Pathology and Neurology at Case Western Reserve University. Dr. Safar is internationally recognized leader in research on neurodegenerative diseases caused by protein misfolding, such as Creutzfeldt-Jakob Disease, mad cow disease (BSE), and Alzheimer’s disease. He has more than two decades of research experience in molecular biology, immunology, biochemistry, and conformation of prion proteins. He discovered previously unknown forms of prions, which led to the findings of new prion diseases, better understanding of the conformation of prion proteins, and unraveling molecular mechanisms of prion strains. Dr. Safar invented the Conformation-Dependent Immunoassay (CDI) for detection and differentiation of infectious isoform of the prion protein (PrPSc). The invention was patented internationally, and the CDI test validated for diagnosis of Creutzfeldt-Jakob Disease, and animal diseases (BSE, scrapie) in European Union. It is a completely new approach for detecting prions and other amyloids, including Alzheimer’s disease.

Dr. Safar earned his MD from the School of Medicine at Charles University, Prague, Czech Republic. He is Board Certified in Neurology, and following his postdoctoral training in biochemistry and neuroscience, and Research Fellowship at the National Institute of Neurological Disorders and Stroke (NINDS), he served as a Senior Scientist in the Laboratory of Central Nervous System Studies at the NINDS Bethesda, Maryland, directed by D.C. Gajdusek (Nobel Prize for Medicine 1976). Here he demonstrated that the difference between the normal and pathogenic prion protein lies in conformational transition, and he introduced the novel concept of the folding intermediate as a crucial stage in prion formation. For this groundbreaking research, he received the National Institutes of Health Award of Merit.

Following his 10-year research appointment at NINDS, Dr. Safar joined the Institute for Neurodegenerative Diseases at the University of California at San Francisco (UCSF), directed by S.B. Prusiner (Nobel Prize for Medicine 1997), and became Associate Professor in the Department of Neurology. During his 12 years at UCSF he expanded his prion research and discovered a new prion protein isoform, and led a research team that developed a preclinical blood test for the detection of human prions.