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CNS & Neurological Disorders - Drug Targets

Editor-in-Chief

ISSN (Print): 1871-5273
ISSN (Online): 1996-3181

The Pathogenic Mechanisms of Prion Diseases

Author(s): T. Voigtlander and U. Unterberger

Volume 6, Issue 6, 2007

Page: [424 - 455] Pages: 32

DOI: 10.2174/187152707783399175

Price: $65

Abstract

Prion diseases are rare fatal neurodegenerative disorders that may either occur sporadically, or be inherited or infectiously acquired in humans. Irrespective of etiology, they can be transmitted to other individuals, this fact being responsible for the public attention prion diseases have received especially since the nineteen nineties, when a new variant of Creutzfeldt-Jakob disease linked to the consumption of prion contaminated beef occurred for the first time in Great Britain. The infectious particle, termed prion, is presumably composed exclusively of a misfolded, partially proteaseresistant conformer (PrPSc) of a normal cell surface protein, the cellular prion protein (PrPC). The pathogenesis of prion diseases comprises entry, spread, and amplification of infectivity in the body periphery in infectiously acquired forms, as well as mechanisms of neuronal cell death in the central nervous system in all disease subtypes. Most experimental therapeutic approaches are either targeted to PrPC or PrPSc, or to the process of conversion from PrPC to PrPSc. Neuroprotective strategies aiming at an interruption of central nervous system pathogenesis have also been tested, albeit with only moderate success. In this review, we discuss actual and potential drug targets in the context of the pathogenic mechanisms of prion diseases.

Keywords: Prions, Creutzfeldt-Jakob disease, scrapie, drug targets, therapy, pathogenesis, stress, neurodegeneration

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