Journal
EXPERT OPINION ON BIOLOGICAL THERAPY
Volume 8, Issue 7, Pages 923-940Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1517/14712598.8.7.923
Keywords
antiprion drugs; prion clearance; prion diseases; prion protein; prion therapy
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Background: Spongiform neurodegeneration is the pathological hallmark of individuals suffering from prion disease. These disorders, whose manifestation is sporadic, familial or acquired by infection, are caused by accumulation of the aberrantly folded isoform of the cellular prion protein (PrPc), termed PrPSc. Although usually rare, prion disorders are inevitably fatal and transferrable by infection. Objective: Pathology is restricted to the central nervous system and premortem diagnosis is usually not possible. Yet, promising approaches towards developing therapeutic regimens have been made recently. Methods: The biology of priori proteins and current models of neurotoxicity are discussed and prophylactic and therapeutic concepts are introduced. Results/condusions: Although various promising drug candidates with antiprion activity have been identified, this proof-of-concept cannot be transferred into translational medicine yet.
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