Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome

Human transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-SträusslerScheinker syndrome (GSS), and fatal familial insomnia. GSS is a genetically determined TSE caused by a range of...

Descripción completa

Detalles Bibliográficos
Autores: Elezgarai, Saioa R., Fernández Borges, Natalia, Eraña, Hasier, Sevillano Mantas, Alejandro Manuel, Charco, Jorge M., Harrathi, Chafik, Saá, Paula, Gil, David, Kong, Qingzhong, Rodríguez Requena, Jesús, Andréoletti, Olivier, Castilla, Joaquín
Tipo de recurso: artículo
Fecha de publicación:2017
País:España
Institución:Universidad de Santiago de Compostela (USC)
Repositorio:Minerva. Repositorio Institucional de la Universidad de Santiago de Compostela
Idioma:inglés
OAI Identifier:oai:minerva.usc.gal:10347/22917
Acceso en línea:http://hdl.handle.net/10347/22917
Access Level:acceso abierto
id ES_3396c207174e0ddcfdbbd6514fb4efbb
oai_identifier_str oai:minerva.usc.gal:10347/22917
network_acronym_str ES
network_name_str España
repository_id_str
spelling Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndromeElezgarai, Saioa R.Fernández Borges, NataliaEraña, HasierSevillano Mantas, Alejandro ManuelCharco, Jorge M.Harrathi, ChafikSaá, PaulaGil, DavidKong, QingzhongRodríguez Requena, JesúsAndréoletti, OlivierCastilla, JoaquínHuman transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-SträusslerScheinker syndrome (GSS), and fatal familial insomnia. GSS is a genetically determined TSE caused by a range of mutations within the prion protein (PrP) gene. Several animal models, based on the expression of PrPs carrying mutations analogous to human heritable prion diseases, support that mutations might predispose PrP to spontaneously misfold. An adapted Protein Misfolding Cyclic Amplification methodology based on the use of human recombinant PrP (recPMCA) generated different self-propagating misfolded proteins spontaneously. These were characterized biochemically and structurally, and the one partially sharing some of the GSS PrPSc molecular features was inoculated into different animal models showing high infectivity. This constitutes an infectious recombinant prion which could be an invaluable model for understanding GSS. Moreover, this study proves the possibility to generate recombinant versions of other human prion diseases that could provide a further understanding on the molecular features of these devastating disorders.Nature Publishing GroupUniversidade de Santiago de Compostela. Centro de Investigación en Medicina Molecular e Enfermidades CrónicasUniversidade de Santiago de Compostela. Departamento de Psiquiatría, Radioloxía, Saúde Pública, Enfermaría e Medicina20172017-01-0120172017-01-01journal articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10347/22917reponame:Minerva. Repositorio Institucional de la Universidad de Santiago de Compostelainstname:Universidad de Santiago de Compostela (USC)Inglésengopen accesshttp://purl.org/coar/access_right/c_abf2© The Author(s) 2017. Open Access. This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as 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 images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/http://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:minerva.usc.gal:10347/229172026-06-15T12:47:27Z
dc.title.none.fl_str_mv Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
title Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
spellingShingle Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
Elezgarai, Saioa R.
title_short Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
title_full Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
title_fullStr Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
title_full_unstemmed Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
title_sort Generation of a new infectious recombinant prion: a model to understand Gerstmann-Straussler-Scheinker syndrome
dc.creator.none.fl_str_mv Elezgarai, Saioa R.
Fernández Borges, Natalia
Eraña, Hasier
Sevillano Mantas, Alejandro Manuel
Charco, Jorge M.
Harrathi, Chafik
Saá, Paula
Gil, David
Kong, Qingzhong
Rodríguez Requena, Jesús
Andréoletti, Olivier
Castilla, Joaquín
author Elezgarai, Saioa R.
author_facet Elezgarai, Saioa R.
Fernández Borges, Natalia
Eraña, Hasier
Sevillano Mantas, Alejandro Manuel
Charco, Jorge M.
Harrathi, Chafik
Saá, Paula
Gil, David
Kong, Qingzhong
Rodríguez Requena, Jesús
Andréoletti, Olivier
Castilla, Joaquín
author_role author
author2 Fernández Borges, Natalia
Eraña, Hasier
Sevillano Mantas, Alejandro Manuel
Charco, Jorge M.
Harrathi, Chafik
Saá, Paula
Gil, David
Kong, Qingzhong
Rodríguez Requena, Jesús
Andréoletti, Olivier
Castilla, Joaquín
author2_role author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universidade de Santiago de Compostela. Centro de Investigación en Medicina Molecular e Enfermidades Crónicas
Universidade de Santiago de Compostela. Departamento de Psiquiatría, Radioloxía, Saúde Pública, Enfermaría e Medicina
description Human transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative disorders that include Kuru, Creutzfeldt-Jakob disease, Gerstmann-SträusslerScheinker syndrome (GSS), and fatal familial insomnia. GSS is a genetically determined TSE caused by a range of mutations within the prion protein (PrP) gene. Several animal models, based on the expression of PrPs carrying mutations analogous to human heritable prion diseases, support that mutations might predispose PrP to spontaneously misfold. An adapted Protein Misfolding Cyclic Amplification methodology based on the use of human recombinant PrP (recPMCA) generated different self-propagating misfolded proteins spontaneously. These were characterized biochemically and structurally, and the one partially sharing some of the GSS PrPSc molecular features was inoculated into different animal models showing high infectivity. This constitutes an infectious recombinant prion which could be an invaluable model for understanding GSS. Moreover, this study proves the possibility to generate recombinant versions of other human prion diseases that could provide a further understanding on the molecular features of these devastating disorders.
publishDate 2017
dc.date.none.fl_str_mv 2017
2017-01-01
2017
2017-01-01
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv http://hdl.handle.net/10347/22917
url http://hdl.handle.net/10347/22917
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
http://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Nature Publishing Group
publisher.none.fl_str_mv Nature Publishing Group
dc.source.none.fl_str_mv reponame:Minerva. Repositorio Institucional de la Universidad de Santiago de Compostela
instname:Universidad de Santiago de Compostela (USC)
instname_str Universidad de Santiago de Compostela (USC)
reponame_str Minerva. Repositorio Institucional de la Universidad de Santiago de Compostela
collection Minerva. Repositorio Institucional de la Universidad de Santiago de Compostela
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869405756591177728
score 15,812455

Similares en LA Referencia