Prion Diseases and Emerging Prion Diseases

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Abstract:

Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are fatal neurodegenerative disorders. An abnormal isoform of the prion protein (PrPSc) generated by post-translational modification of the cellular prion protein (PrPC) is believed to be the main component of this infectious agent. PrPSc is relatively resistant to proteinase K (PK) digestion. This characteristic has been widely accepted as the physicochemical basis for distinguishing between PrPC and PrPSc. PrPC is a glycoprotein that contains 2 Asnlinked glycosylation sites; it is present in the cells in 3 different glycoforms, including an unglycosylated form. Hence, for different prion strains, PrPSc exhibits different glycoform patterns with different ratios of the 3 forms by western blot. Recently, phenotypes of TSEs have emerged that exhibit PrPSc with different glycoform patterns and/or mild PK resistance in comparison with previously described typical cases. Regarding sheep scrapie, atypical scrapie cases that are represented by Nor98 have been reported among sheep previously presumed to be genetically scrapie-resistant. Moreover, atypical bovine spongiform encephalopathy (BSE) cases have been reported. These are classified into 2 phenotypes (H-type and L-type) based on the molecular weight of unglycosylated band of PK-digested PrPSc. The origin of these emerging prion diseases is obscure, conformational differences of PrPSc may cause the different biological and biochemical characteristics of prion strains.

Document Type: Research Article

DOI: http://dx.doi.org/10.2174/092986708783955437

Affiliations: Prion Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba, Ibaraki 305-0856, Japan.

Publication date: April 1, 2008

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  • Current Medicinal Chemistry covers all the latest and outstanding developments in medicinal chemistry and rational drug design. Each issue contains a series of timely in-depth reviews written by leaders in the field covering a range of the current topics in medicinal chemistry. Current Medicinal Chemistry is an essential journal for every medicinal chemist who wishes to be kept informed and up-to-date with the latest and most important developments.
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