Creutzfeldt-Jakob disease: current issues (review)

Abstract

Creutzfeldt-Jakob disease and others human prion diseases arefatal neurodegenerative conditions. Etiologic classificationincludes sporadic, hereditary and acquired forms.Conformational change of the normal (cellular) form of prionprotein (PrPc) to a pathological form (PrPSс) is considered centralto formation of the infectious agent. In this article themolecular classification of sporadic CJD, the phenotypic variabilityand the major pathogenetic pathways in prion diseaseshave been analyzed. The unique resistance of prions to classicmethods of decontamination, and evidence that prion diseasescan be transmitted iatrogenically pose a serious control todecontamination procedures. Many therapeutic strategies havebeen tested as potential treatments for prion diseases in cell culturesand in animals. But only few trials of human prion diseasehave been published or ongoing.

Keywords

• creutzfeldt-jakob disease
• prion