Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.

Chronic wasting disease is a transmissible spongiform encephalopathy of cervids. This fatal neurodegenerative disease is caused by misfolding of the cellular prion protein (PrPC) to pathogenic conformers (PrPSc), and the pathogenic forms accumulate in the brain and other tissues. Real-time Quaking I...

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Main Authors: Soyoun Hwang, Justin J Greenlee, Eric M Nicholson
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2020-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0227487
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author Soyoun Hwang
Justin J Greenlee
Eric M Nicholson
author_facet Soyoun Hwang
Justin J Greenlee
Eric M Nicholson
author_sort Soyoun Hwang
collection DOAJ
description Chronic wasting disease is a transmissible spongiform encephalopathy of cervids. This fatal neurodegenerative disease is caused by misfolding of the cellular prion protein (PrPC) to pathogenic conformers (PrPSc), and the pathogenic forms accumulate in the brain and other tissues. Real-time Quaking Induced Conversion (RT-QuIC) can be used for the detection of prions and for prion strain discrimination in a variety of biological tissues from humans and animals. In this study, we evaluated how either PrPSc from cervids of different genotypes or PrPSc from different sources of CWD influence the fibril formation of recombinant bank vole (BV) or human prion proteins using RT-QuIC. We found that reaction mixtures seeded with PrPSc from different genotypes of white-tailed deer or reindeer brains have similar conversion efficiency with both substrates. Also, we observed similar results when assays were seeded with different sources of CWD. Thus, we conclude that the genotypes of all sources of CWD used in this study do not influence the level of conversion of PrPC to PrPSc.
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spelling doaj.art-81853356354c4cfaaa0a9dce2f21bedb2022-12-21T22:38:54ZengPublic Library of Science (PLoS)PLoS ONE1932-62032020-01-01151e022748710.1371/journal.pone.0227487Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.Soyoun HwangJustin J GreenleeEric M NicholsonChronic wasting disease is a transmissible spongiform encephalopathy of cervids. This fatal neurodegenerative disease is caused by misfolding of the cellular prion protein (PrPC) to pathogenic conformers (PrPSc), and the pathogenic forms accumulate in the brain and other tissues. Real-time Quaking Induced Conversion (RT-QuIC) can be used for the detection of prions and for prion strain discrimination in a variety of biological tissues from humans and animals. In this study, we evaluated how either PrPSc from cervids of different genotypes or PrPSc from different sources of CWD influence the fibril formation of recombinant bank vole (BV) or human prion proteins using RT-QuIC. We found that reaction mixtures seeded with PrPSc from different genotypes of white-tailed deer or reindeer brains have similar conversion efficiency with both substrates. Also, we observed similar results when assays were seeded with different sources of CWD. Thus, we conclude that the genotypes of all sources of CWD used in this study do not influence the level of conversion of PrPC to PrPSc.https://doi.org/10.1371/journal.pone.0227487
spellingShingle Soyoun Hwang
Justin J Greenlee
Eric M Nicholson
Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
PLoS ONE
title Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
title_full Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
title_fullStr Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
title_full_unstemmed Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
title_short Role of donor genotype in RT-QuIC seeding activity of chronic wasting disease prions using human and bank vole substrates.
title_sort role of donor genotype in rt quic seeding activity of chronic wasting disease prions using human and bank vole substrates
url https://doi.org/10.1371/journal.pone.0227487
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