Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.

<h4>Objective</h4>The glycoprofile of pathological prion protein (PrP(res)) is widely used as a diagnosis marker in Creutzfeldt-Jakob disease (CJD) and is thought to vary in a strain-specific manner. However, that the same glycoprofile of PrP(res) always accumulates in the whole brain of...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Etienne Levavasseur, Isabelle Laffont-Proust, Emilie Morain, Baptiste A Faucheux, Nicolas Privat, Katell Peoc'h, Véronique Sazdovitch, Jean-Philippe Brandel, Jean-Jacques Hauw, Stéphane Haïk
Formato: article
Lenguaje:EN
Publicado: Public Library of Science (PLoS) 2008
Materias:
R
Q
Acceso en línea:https://doaj.org/article/7bc2423300c54e9fbbe5a6970abf32ec
Etiquetas: Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
id oai:doaj.org-article:7bc2423300c54e9fbbe5a6970abf32ec
record_format dspace
spelling oai:doaj.org-article:7bc2423300c54e9fbbe5a6970abf32ec2021-11-25T06:11:27ZRegulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.1932-620310.1371/journal.pone.0002786https://doaj.org/article/7bc2423300c54e9fbbe5a6970abf32ec2008-07-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/18665216/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Objective</h4>The glycoprofile of pathological prion protein (PrP(res)) is widely used as a diagnosis marker in Creutzfeldt-Jakob disease (CJD) and is thought to vary in a strain-specific manner. However, that the same glycoprofile of PrP(res) always accumulates in the whole brain of one individual has been questioned. We aimed to determine whether and how PrP(res) glycosylation is regulated in the brain of patients with sporadic and variant Creutzfeldt-Jakob disease.<h4>Methods</h4>PrP(res) glycoprofiles in four brain regions from 134 patients with sporadic or variant CJD were analyzed as a function of the genotype at codon 129 of PRNP and the Western blot type of PrP(res).<h4>Results</h4>The regional distribution of PrP(res) glycoforms within one individual was heterogeneous in sporadic but not in variant CJD. PrP(res) glycoforms ratio significantly correlated with the genotype at codon 129 of the prion protein gene and the Western blot type of PrP(res) in a region-specific manner. In some cases of sCJD, the glycoprofile of thalamic PrP(res) was undistinguishable from that observed in variant CJD.<h4>Interpretation</h4>Regulations leading to variations of PrP(res) pattern between brain regions in sCJD patients, involving host genotype and Western blot type of PrP(res) may contribute to the specific brain targeting of prion strains and have direct implications for the diagnosis of the different forms of CJD.Etienne LevavasseurIsabelle Laffont-ProustEmilie MorainBaptiste A FaucheuxNicolas PrivatKatell Peoc'hVéronique SazdovitchJean-Philippe BrandelJean-Jacques HauwStéphane HaïkPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 3, Iss 7, p e2786 (2008)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Etienne Levavasseur
Isabelle Laffont-Proust
Emilie Morain
Baptiste A Faucheux
Nicolas Privat
Katell Peoc'h
Véronique Sazdovitch
Jean-Philippe Brandel
Jean-Jacques Hauw
Stéphane Haïk
Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
description <h4>Objective</h4>The glycoprofile of pathological prion protein (PrP(res)) is widely used as a diagnosis marker in Creutzfeldt-Jakob disease (CJD) and is thought to vary in a strain-specific manner. However, that the same glycoprofile of PrP(res) always accumulates in the whole brain of one individual has been questioned. We aimed to determine whether and how PrP(res) glycosylation is regulated in the brain of patients with sporadic and variant Creutzfeldt-Jakob disease.<h4>Methods</h4>PrP(res) glycoprofiles in four brain regions from 134 patients with sporadic or variant CJD were analyzed as a function of the genotype at codon 129 of PRNP and the Western blot type of PrP(res).<h4>Results</h4>The regional distribution of PrP(res) glycoforms within one individual was heterogeneous in sporadic but not in variant CJD. PrP(res) glycoforms ratio significantly correlated with the genotype at codon 129 of the prion protein gene and the Western blot type of PrP(res) in a region-specific manner. In some cases of sCJD, the glycoprofile of thalamic PrP(res) was undistinguishable from that observed in variant CJD.<h4>Interpretation</h4>Regulations leading to variations of PrP(res) pattern between brain regions in sCJD patients, involving host genotype and Western blot type of PrP(res) may contribute to the specific brain targeting of prion strains and have direct implications for the diagnosis of the different forms of CJD.
format article
author Etienne Levavasseur
Isabelle Laffont-Proust
Emilie Morain
Baptiste A Faucheux
Nicolas Privat
Katell Peoc'h
Véronique Sazdovitch
Jean-Philippe Brandel
Jean-Jacques Hauw
Stéphane Haïk
author_facet Etienne Levavasseur
Isabelle Laffont-Proust
Emilie Morain
Baptiste A Faucheux
Nicolas Privat
Katell Peoc'h
Véronique Sazdovitch
Jean-Philippe Brandel
Jean-Jacques Hauw
Stéphane Haïk
author_sort Etienne Levavasseur
title Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
title_short Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
title_full Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
title_fullStr Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
title_full_unstemmed Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
title_sort regulating factors of prp glycosylation in creutzfeldt-jakob disease--implications for the dissemination and the diagnosis of human prion strains.
publisher Public Library of Science (PLoS)
publishDate 2008
url https://doaj.org/article/7bc2423300c54e9fbbe5a6970abf32ec
work_keys_str_mv AT etiennelevavasseur regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT isabellelaffontproust regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT emiliemorain regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT baptisteafaucheux regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT nicolasprivat regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT katellpeoch regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT veroniquesazdovitch regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT jeanphilippebrandel regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT jeanjacqueshauw regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
AT stephanehaik regulatingfactorsofprpglycosylationincreutzfeldtjakobdiseaseimplicationsforthedisseminationandthediagnosisofhumanprionstrains
_version_ 1718414051279634432