Afficher la couverture 'Prions from Sporadic Creutzfeldt-Jakob Disease Patients Propagate as Strain Mixtures | Cassard, Hervé' en grand format
/5

0 avis

Prions from Sporadic Creutzfeldt-Jakob Disease Patients Propagate as Strain Mixtures

Archive ouverte

Cassard, Hervé | Huor, Alvina | Espinosa, Juan-Carlos | Douet, Jean-Yves | Lugan, Severine | Aron, Naima | Vilette, Didier | Delisle, Marie-Bernadette | Marín-Moreno, Alba | Peran, Patrice | Beringue, Vincent | Torres, Juan Maria | Ironside, James | Andreoletti, Olivier

Edité par CCSD ; American Society for Microbiology -

International audience. sCJD occurrence is currently assumed to result from spontaneous and stochastic formation of a misfolded PrP nucleus in the brains of affected patients. This original nucleus then recruits and converts nascent PrP C into PrP Sc , leading to the propagation of prions in the patient’s brain. Our study demonstrates the coexistence of two prion strains in the brains of a majority of the 23 sCJD patients investigated. The relative proportion of these sCJD strains varied both between patients and between brain areas in a single patient. These findings strongly support the view that the replication of an sCJD prion strain in the brain of a patient can result in the propagation of different prion strain subpopulations. Beyond its conceptual importance for our understanding of prion strain properties and evolution, the sCJD strain mixture phenomenon and its frequency among patients have important implications for the development of therapeutic strategies for prion diseases.

Suggestions

Du même auteur

Prions from Sporadic Creutzfeldt-Jakob Disease Patients Propagate as Strain Mixtures

Archive ouverte | Cassard, Hervé | CCSD

International audience. Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently classified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K-digested abnormal p...

Allelic Interference in Prion Replication Is Modulated by the Convertibility of the Interfering PrP C and Other Host-Specific Factors

Archive ouverte | Espinosa, Juan Carlos | CCSD

International audience. Prion propagation can be interfered with by the expression of a second prion protein in the host. In the present study, we investigated prion propagation in a host expressing two different pr...

Wide distribution of prion infectivity in the peripheral tissues of vCJD and sCJD patients

Archive ouverte | Douet, Jean-Yves | CCSD

International audience. Sporadic Creutzfeldt-Jakob disease (sCJD) is the commonest human prion disease, occurring most likely as the consequence of spontaneous formation of abnormal prion protein in the central nerv...

Chargement des enrichissements...