Strain variation in treatment and prevention of human prion diseases.

Autor:	Ellett LJ; Department of Microbiology and Immunology, The University of Melbourne, Parkville, VIC, Australia., Revill ZT; Department of Microbiology and Immunology, The University of Melbourne, Parkville, VIC, Australia., Koo YQ; Department of Microbiology and Immunology, The University of Melbourne, Parkville, VIC, Australia., Lawson VA; Department of Microbiology and Immunology, The University of Melbourne, Parkville, VIC, Australia. Electronic address: v.lawson@unimelb.edu.au.
Jazyk:	angličtina
Zdroj:	Progress in molecular biology and translational science [Prog Mol Biol Transl Sci] 2020; Vol. 175, pp. 121-145. Date of Electronic Publication: 2020 Sep 11.
DOI:	10.1016/bs.pmbts.2020.08.006
Abstrakt:	Transmissible spongiform encephalopathies or prion diseases describe a number of different human disorders that differ in their clinical phenotypes, which are nonetheless united by their transmissible nature and common pathology. Clinical variation in the absence of a conventional infectious agent is believed to be encoded by different conformations of the misfolded prion protein. This misfolded protein is the target of methods designed to prevent disease transmission in a surgical setting and reduction of the misfolded seed or preventing its continued propagation have been the focus of therapeutic strategies. It is therefore possible that strain variation may influence the efficacy of prevention and treatment approaches. Historically, an understanding of prion disease transmission and pathogenesis has been focused on research tools developed using agriculturally relevant strains of prion disease. However, an increased understanding of the molecular biology of human prion disorders has highlighted differences not only between different forms of the disease affecting humans and animals but also within diseases such as Creutzfeldt-Jakob Disease (CJD), which is represented by several sporadic CJD specific conformations and an additional conformation associated with variant CJD. In this chapter we will discuss whether prion strain variation can affect the efficacy of methods used to decontaminate prions and whether strain variation in pre-clinical models of prion disease can be used to identify therapeutic strategies that have the best possible chance of success in the clinic. (© 2020 Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu