Creutzfeldt-Jakob disease: current issues (review)

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Abstract

Creutzfeldt-Jakob disease and others human prion diseases are fatal neurodegenerative conditions. Etiologic classification includes sporadic, hereditary and acquired forms. Conformational change of the normal (cellular) form of prion protein (PrPc) to a pathological form (PrPSс) is considered central to formation of the infectious agent. In this article the molecular classification of sporadic CJD, the phenotypic variability and the major pathogenetic pathways in prion diseases have been analyzed. The unique resistance of prions to classic methods of decontamination, and evidence that prion diseases can be transmitted iatrogenically pose a serious control to decontamination procedures. Many therapeutic strategies have been tested as potential treatments for prion diseases in cell cultures and in animals. But only few trials of human prion disease have been published or ongoing.

 

About the authors

A. V. Peresedova

Research Center of Neurology, Russian Academy of Medical Sciences

Author for correspondence.
Email: platonova@neurology.ru
Russian Federation, Moscow

I. A. Zavalishin

Research Center of Neurology, Russian Academy of Medical Sciences

Email: platonova@neurology.ru
Russian Federation, Moscow

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Copyright (c) 2012 Peresedova A.V., Zavalishin I.A.

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