Cargando…
Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice
Host prion (PrP(C)) genotype is a major determinant for the susceptibility to prion diseases. The Q/K(222)-PrP(C) polymorphic variant provides goats and mice with high resistance against classical scrapie and bovine spongiform encephalopathy (BSE); yet its effect against atypical scrapie is unknown....
Autores principales: | , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5034450/ https://www.ncbi.nlm.nih.gov/pubmed/27659200 http://dx.doi.org/10.1186/s13567-016-0380-7 |
_version_ | 1782455271170244608 |
---|---|
author | Aguilar-Calvo, Patricia Espinosa, Juan-Carlos Andréoletti, Olivier González, Lorenzo Orge, Leonor Juste, Ramón Torres, Juan-María |
author_facet | Aguilar-Calvo, Patricia Espinosa, Juan-Carlos Andréoletti, Olivier González, Lorenzo Orge, Leonor Juste, Ramón Torres, Juan-María |
author_sort | Aguilar-Calvo, Patricia |
collection | PubMed |
description | Host prion (PrP(C)) genotype is a major determinant for the susceptibility to prion diseases. The Q/K(222)-PrP(C) polymorphic variant provides goats and mice with high resistance against classical scrapie and bovine spongiform encephalopathy (BSE); yet its effect against atypical scrapie is unknown. Here, transgenic mice expressing the goat wild-type (wt) or the K(222)-PrP(C) variant were intracerebrally inoculated with several natural cases of atypical scrapie from sheep and goat and their susceptibility to the prion disease was determined. Goat wt and K(222)-PrP(C) transgenic mice were 100% susceptible to all the atypical scrapie isolates, showing similar survival times and almost identical disease phenotypes. The capacity of the K(222)-PrP(C) variant to replicate specifically the atypical scrapie strain as efficiently as the goat wt PrP(C), but not the classical scrapie or cattle-BSE as previously reported, further suggests the involvement of concrete areas of the host PrP(C) in the strain-dependent replication of prions. |
format | Online Article Text |
id | pubmed-5034450 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-50344502016-09-29 Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice Aguilar-Calvo, Patricia Espinosa, Juan-Carlos Andréoletti, Olivier González, Lorenzo Orge, Leonor Juste, Ramón Torres, Juan-María Vet Res Research Article Host prion (PrP(C)) genotype is a major determinant for the susceptibility to prion diseases. The Q/K(222)-PrP(C) polymorphic variant provides goats and mice with high resistance against classical scrapie and bovine spongiform encephalopathy (BSE); yet its effect against atypical scrapie is unknown. Here, transgenic mice expressing the goat wild-type (wt) or the K(222)-PrP(C) variant were intracerebrally inoculated with several natural cases of atypical scrapie from sheep and goat and their susceptibility to the prion disease was determined. Goat wt and K(222)-PrP(C) transgenic mice were 100% susceptible to all the atypical scrapie isolates, showing similar survival times and almost identical disease phenotypes. The capacity of the K(222)-PrP(C) variant to replicate specifically the atypical scrapie strain as efficiently as the goat wt PrP(C), but not the classical scrapie or cattle-BSE as previously reported, further suggests the involvement of concrete areas of the host PrP(C) in the strain-dependent replication of prions. BioMed Central 2016-09-22 2016 /pmc/articles/PMC5034450/ /pubmed/27659200 http://dx.doi.org/10.1186/s13567-016-0380-7 Text en © The Author(s) 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Article Aguilar-Calvo, Patricia Espinosa, Juan-Carlos Andréoletti, Olivier González, Lorenzo Orge, Leonor Juste, Ramón Torres, Juan-María Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title | Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title_full | Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title_fullStr | Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title_full_unstemmed | Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title_short | Goat K(222)-PrP(C) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
title_sort | goat k(222)-prp(c) polymorphic variant does not provide resistance to atypical scrapie in transgenic mice |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5034450/ https://www.ncbi.nlm.nih.gov/pubmed/27659200 http://dx.doi.org/10.1186/s13567-016-0380-7 |
work_keys_str_mv | AT aguilarcalvopatricia goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT espinosajuancarlos goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT andreolettiolivier goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT gonzalezlorenzo goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT orgeleonor goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT justeramon goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice AT torresjuanmaria goatk222prpcpolymorphicvariantdoesnotprovideresistancetoatypicalscrapieintransgenicmice |