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.
Current Medicinal Chemistry
Title: Prion Diseases and Emerging Prion Diseases
Volume: 15 Issue: 9
Author(s): Takashi Yokoyama and Shirou Mohri
Affiliation:
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.
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Cite this article as:
Yokoyama Takashi and Mohri Shirou, Prion Diseases and Emerging Prion Diseases, Current Medicinal Chemistry 2008; 15 (9) . https://dx.doi.org/10.2174/092986708783955437
DOI https://dx.doi.org/10.2174/092986708783955437 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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