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Prion Protein-Specific Antibodies-Development, Modes of Action and Therapeutics Application

Prion Biology Laboratory, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste 34100, Italy
Author to whom correspondence should be addressed.
Viruses 2014, 6(10), 3719-3737;
Received: 31 July 2014 / Revised: 22 September 2014 / Accepted: 23 September 2014 / Published: 1 October 2014
(This article belongs to the Special Issue Recent Developments in the Prion Field)
Prion diseases or Transmissible Spongiform Encephalopathies (TSEs) are lethal neurodegenerative disorders involving the misfolding of the host encoded cellular prion protein, PrPC. This physiological form of the protein is expressed throughout the body, and it reaches the highest levels in the central nervous system where the pathology occurs. The conversion into the pathogenic isoform denoted as prion or PrPSc is the key event in prion disorders. Prominent candidates for the treatment of prion diseases are antibodies and their derivatives. Anti-PrPC antibodies are able to clear PrPSc from cell culture of infected cells. Furthermore, application of anti-PrPC antibodies suppresses prion replication in experimental animal models. Major drawbacks of immunotherapy are immune tolerance, the risks of neurotoxic side effects, limited ability of compounds to cross the blood-brain barrier and their unfavorable pharmacokinetic. The focus of this review is to recapitulate the current understanding of the molecular mechanisms for antibody mediated anti-prion activity. Although relevant for designing immunotherapeutic tools, the characterization of key antibody parameters shaping the molecular mechanism of the PrPC to PrPSc conversion remains elusive. Moreover, this review illustrates the various attempts towards the development of anti-PrP antibody compounds and discusses therapeutic candidates that modulate PrP expression. View Full-Text
Keywords: prion; PrP; antibody; recombinant antibody; immunotherapy; molecular mechanism prion; PrP; antibody; recombinant antibody; immunotherapy; molecular mechanism
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Rovis, T.L.; Legname, G. Prion Protein-Specific Antibodies-Development, Modes of Action and Therapeutics Application. Viruses 2014, 6, 3719-3737.

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