Review
Update on human prion disease

https://doi.org/10.1016/j.bbadis.2007.02.010Get rights and content
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Abstract

The recognition that variant Creutzfeldt–Jakob disease (vCJD) is caused by the same prion strain as bovine spongiform encephalopathy in cattle has dramatically highlighted the need for a precise understanding of the molecular biology of human prion diseases. Detailed clinical, pathological and molecular data from a large number of human prion disease patients indicate that phenotypic diversity in human prion disease relates in part to the propagation of disease-related PrP isoforms with distinct physicochemical properties. Incubation periods of prion infection in humans can exceed 50 years and therefore it will be some years before the extent of any human vCJD epidemic can be predicted with confidence.

Keywords

Bovine spongiform encephalopathy
Creutzfeldt-Jakob disease
Fatal familial insomnia
Gerstmann-Sträussler-Scheinker disease
Kuru
Prion
Prion disease
Prion protein
Transmissible spongiform encephalopathy
Variant Creutzfeldt-Jakob disease

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