Advances in the development of antibody-based immunotherapy against prion disease
Huiying Gu,1 Richard C Dodel,2 Martin R Farlow,1 Yansheng Du1
1Department of Neurology, School of Medicine, Indiana University, Indianapolis, IN, USA; 2Department of Neurology, Philipps University, Marburg, Germany
Abstract: Prion disease, also known as transmissible spongiform encephalopathies, is the name given to a group of neurodegenerative disorders. Transformation of the cellular prion protein (PrPC) into self-replicating and proteinase K-resistant PrP (PrPSc) in the brain is the pathological hallmark of the disease. All prion disorders have a rapidly progressive and lethal course after onset, and no effective therapy currently exists. Antibody-based immunotherapy has been extensively investigated in neurodegenerative disorders associated with protein misfolding, including prion disease. This review summarizes and outlines the developments in and limitations of active and passive immunization approaches to the prevention and treatment for prion disease. In addition, the potential of these therapeutic strategies is discussed.
Keywords: transmissible spongiform encephalopathies, neurodegenerative, prion protein, proteinase K-resistant PrP
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