Review Nature Reviews Neurology, advance online publication, Published
online 18 December 2012 | doi:10.1038/nrneurol.2012.258
Subject Categories: Spongiform encephalopathies | Neurodegenerative
disease
Immunotherapy in prion disease
Yvonne Roettger, Yansheng Du, Michael Bacher, Inga Zerr, Richard Dodel
& Jan-Philipp Bach About the authors
Top of page Abstract Transmissible spongiform encephalopathies (TSEs), also
known as prion diseases, describe a group of fatal neurodegenerative disorders
affecting both humans and animals. Accumulation of misfolded prion proteins is
the pathological hallmark of these disorders; such accumulation occurs in
lymphoreticular tissue prior to CNS involvement in scrapie, experimental models
and human variant Creutzfeldt–Jakob disease. Lymphoreticular accumulation of
misfolded prion protein has not been demonstrated in human sporadic or genetic
forms of TSE. Once clinical symptoms develop, all prion disorders have a rapidly
progressive and lethal course, and no effective therapy exists. In the past 10
years, antibody-based immunotherapy has been considered for other
neurodegenerative disorders associated with protein misfolding and, therefore,
might also be an effective approach to prevention or treatment of prion disease.
Self-tolerance to endogenous prion protein is, however, a major challenge to the
development of effective immunotherapy, as is the risk of adverse effects from
active immunization. This Review summarizes the evidence that immunization could
slow disease progression or increase lifespan in animal models of prion
diseases. The therapeutic potential of these strategies in treating patients
with prion diseases is also discussed.
Top of page Author affiliations Y. Roettger, Y. Du, M. Bacher, I. Zerr, R.
Dodel & J. -P. Bach Department of Neurology, Philipps-University Marburg,
Baldingerstrasse, 35043 Marburg, Germany (Y. Roettger, R. Dodel, J. -P. Bach).
Department of Neurology, Indiana University School of Medicine, 975 West Walnut
Street IB 457, Indianapolis, IN 46202, USA (Y. Du). Institute of Immunology,
University of Marburg, Hans-Meerwein-Strasse, 35043 Marburg, Germany (M.
Bacher). National Reference Centre for Transmissible Spongiform
Encephalopathies, Department of Neurology, Georg-August-University Göttingen,
Robert-Koch-Strasse 40, 37075 Göttingen, Germany (I. Zerr).
Correspondence to: R. Dodel dodel@med.uni-marburg.de
Published online 18 December 2012
December 13, 2012
The rise and fall of pentosan polysulfate in prion disease
TSS
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