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Advances in the development of antibody-based immunotherapy against prion disease

Authors Gu H, Dodel R, Farlow MR, Du Y

Received 11 March 2014

Accepted for publication 19 May 2014

Published 30 August 2014 Volume 2014:4 Pages 45—55


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4

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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