Abstract
Transmissible spongiform encepahalopathies (TSEs) are fatal diseases that damage the central nervous system. TSEs are unique in that they may be inherited, infectious or spontaneous. The central pathogenic agent is thought to be a conformationally distinct form (PrPSc) of the endogenous prion protein(PrPc), which is high in beta-sheet content and is resistant to proteases; infectivity is thought to involve formation of PrPSc via imprinting of abnormal conformation on the normal form of the protein (PrPc) by seeds of PrPSc. A number of compounds found to inhibit the conversion of PrPc to PrPSc have been proposed as therapeutics to halt TSEs.
Keywords: spongiform encephalopathies, prions, beta-sheet, proline peptides
Protein & Peptide Letters
Title: Experimental Approaches to Tse Prevention Via Inhibition of Prion Formation
Volume: 11 Issue: 3
Author(s): Galatia Politopoulou
Affiliation:
Keywords: spongiform encephalopathies, prions, beta-sheet, proline peptides
Abstract: Transmissible spongiform encepahalopathies (TSEs) are fatal diseases that damage the central nervous system. TSEs are unique in that they may be inherited, infectious or spontaneous. The central pathogenic agent is thought to be a conformationally distinct form (PrPSc) of the endogenous prion protein(PrPc), which is high in beta-sheet content and is resistant to proteases; infectivity is thought to involve formation of PrPSc via imprinting of abnormal conformation on the normal form of the protein (PrPc) by seeds of PrPSc. A number of compounds found to inhibit the conversion of PrPc to PrPSc have been proposed as therapeutics to halt TSEs.
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Cite this article as:
Politopoulou Galatia, Experimental Approaches to Tse Prevention Via Inhibition of Prion Formation, Protein & Peptide Letters 2004; 11 (3) . https://dx.doi.org/10.2174/0929866043407192
DOI https://dx.doi.org/10.2174/0929866043407192 |
Print ISSN 0929-8665 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5305 |
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