prion disease
Prolongation of prion disease-associated symptomatic phase relates to CD3+ T cell recruitment into the CNS in murine scrapie-infected mice.
... The prion disease natural history includes a long and asymptomatic incubation period (time from 139A inoculation to symptoms onset) and a clinical phase (time from symptoms on- set to ...
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Genetic Testing in Prion Disease: Psychological Consequences of the Decisions to Know or Not to Know
... Living With the Risk of Genetic Prion Disease Schwartz et al. are memory impairment and confusion, followed by ataxia and myoclonus. The course from onset to death ranges from a few months to 5 years. At ...
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Double-Edge Sword of Sustained ROCK Activation in Prion Diseases through Neuritogenesis Defects and Prion Accumulation
... familial prion disease (Fukuoka-1, Fk) [ 23 ...that prion infection dis- turbs all neurotransmitter-associated functions in prion-infected 1C11 5-HT neuronal cells and triggers the production ...
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Context Dependent Neuroprotective Properties of Prion Protein (Prp)
... Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis, Huntington’s disease (HD), and several other more rare conditions, such as prion ...
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Bimodality, prion aggregates infectivity and prediction of strain phenomenon
... of prion replication have been proposed [8, 9, 10, ...of prion disease can be explained by purely physico-chemical mechanisms, as supposed by the protein-only ...of prion aggregates in brain, ...
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Getting to the core of prion superstructural variability
... In prion diseases, the prion protein (PrP C ) misfolds and assembles into various self-propa- gating quaternary structures with predomi- nantly b sheet secondary structure, designated PrP Sc (Sc for ...
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Analyse de l'agrégation des protéines dans les maladies neurodégénératives amyloïdes : application aux maladies à prion
... modified prion structure is responsible for trans- mitted encephalopathies such as vCJD or variant Creutzfeldt-Jakob disease, the disease strongly linked to bovine spongiform encephalopathy (BSE) re- ...
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Highly efficient prion transmission by blood transfusion
... transmitted prion disease with 100% efficiency thereby displaying greater virulence than the transfusion of 200 mL of normal blood spiked with brain homogenate material containing 10 3 ID 50 as measured by ...
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The Shape of the Polymerization Rate in the Prion Equation
... states. Prion strains are characterized by their incubation period (time elapsed between experimental inoculation of the PrPsc infectious agent and clinical onset of the disease) and their lesion profiles ...
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Étude numérique des premières étapes d'agrégation du peptide amyloïde GNNQQNY, impliqué dans une maladie à prion
... 39 • JNL était responsable de rassembler les travaux des trois groupes de recherches. 4.3 Author summary The formation of amyloid fibrils is associated with many neurodegenerative diseases such as Alzheimer’s, ...
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Pressure Reveals Unique Conformational Features in Prion Protein Fibril Diversity
... model system where the structural/functional properties of these PrP assemblies can be elucidated. Knowing how PrP fibril diversity is related to the quaternary structure could aid in understanding how multiple ...
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Prion Amyloid Polymorphs -The Tag Might Change It All
... While the poly-His tag affected marginally the structure of Sup35pNM fibrils (untagged protein) when placed on the N-terminal end of the protein, its positioning at the C-terminal end in contrast radically changed the ...
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Blessings in disguise: biological benefits of prion-like mechanisms
... habitat. A recent study surveying 700 wild S. cerevisiae isolates found that prions were present 210 in at least one third of the strains [19]. Prion loss was induced by transiently inhibiting a 211 ...
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Global stability for the prion equation with general incidence
... normally shaped cellular prion protein, the PrP c . The so-called nucleated polymerization was proposed by [16] as a conversion mechanism of PrP c into PrP Sc . According to this theory the PrP Sc is in a ...
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De l'hypothèse propagation de type prion aux stratégies thérapeutiques d'immunothérapie anti-tau
... heimer’s disease, progressive supranuclear palsy, and argyrophilic grain ...the prion-like tau propagation has been extended from Alzheimer’s disease to ...Alzheimer’s disease, the progression ...
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Analyse de la disposition de la protéine du prion recombinante ovine administrée par voie intraveineuse chez la brebis
... protéine prion est coaté au fond des ...anti-protéine prion (reconnaissant un épitope différent du premier anticorps), couplé à l'acétyl choline estérase est ...
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The prion protein family: a view from the placenta
... panel of created animal models with altered expression of these genes would reveal such roles. It was also suggested that these proteins, which have diverse and large interactomes, may participate to versatile signaling ...
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Contrôler l’équilibre redox cellulaire : une fonction ubiquitaire pour la protéine prion cellulaire ?
... Jakob chez l’homme - est composé essentiellement, voire exclusivement, d’une protéine nommée PrP Sc pour forme scrapie (Sc) de la protéine prion cellu- laire ou PrP C . Ces deux isoformes possè- dent la même ...
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La protéine Prion cellulaire (PrPC) dans la mucoviscidose : Rôle dans le maintien de la barrière épithéliale bronchique
... To shed light on the potential involvement of PrP C in CF, we first assessed its level of expression and characterized its tissue distribution and localization in human bronchi of healthy and CF patients. Using RT-qPCR, ...
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Evaluation expérimentale du risque prion lié aux porteurs asymptomatiques chez l'Homme et le macaque
... of prion replication in the lymphoid organs of vCJD- infected cynomolgus macaques sampled at the terminal stage of the disease, without identifying an obvious correlation with the anatomical sites, the ...
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