Evaluation of the protection of primates transfused with variant Creutzfeldt‐Jakob disease–infected blood products filtered with prion removal devices: a 5‐year update

Lescoutra-Etchegaray, Nathalie; Jaffré, Nina; Sumian, Chryslain; Durand, Valérie; Correia, Evelyne; Mikol, Jacqueline; Luccantoni-Freire, Sophie; Culeux, Audrey; Deslys, Jean‐Philippe; Comoy, Emmanuel

doi:10.1111/trf.12999

Cited by 14 publications

(9 citation statements)

References 25 publications

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“…BSE transmits efficiently to cynomolgous macaques by intracerebral and intravenous routes (including blood transfusion), producing similar clinical and neuropathological features to those observed in human patients affected by vCJD . The relative efficacy of C‐BSE transmission observed in macaques exposed by the oral route to low infectious doses of C‐BSE also fits well with the limited number of vCJD cases that occurred in dietary‐exposed human populations .…”

Section: Classical Bse and Variant Cjdsupporting

confidence: 65%

Animal prion diseases: the risks to human health

Houston

Andréoletti

2019

Brain Pathology

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Transmissible spongiform encephalopathies (TSEs) or prion diseases of animals notably include scrapie in small ruminants, chronic wasting disease (CWD) in cervids and classical bovine spongiform encephalopathy (C-BSE). As the transmission barrier phenomenon naturally limits the propagation of prions from one species to another, and the lack of epidemiological evidence for an association with human prion diseases, the zoonotic potential of these diseases was for a long time considered negligible. However, in 1996, C-BSE was recognized as the cause of a new human prion disease, variant Creutzfeldt-Jakob disease (vCJD), which triggered an unprecedented public health crisis in Europe. Large-scale epidemio-surveillance programs for scrapie and C-BSE that were implemented in the EU after the BSE crisis revealed that the distribution and prevalence of prion diseases in the ruminant population had previously been underestimated. They also led to the recognition of new forms of TSEs (named atypical) in cattle and small ruminants and to the recent identification of CWD in Europe. At this stage, the characterization of the strain diversity and zoonotic abilities associated with animal prion diseases remains largely incomplete. However, transmission experiments in nonhuman primates and transgenic mice expressing human PrP clearly indicate that classical scrapie, and certain forms of atypical BSE (L-BSE) or CWD may have the potential to infect humans. The remaining uncertainties about the origins and relationships between animal prion diseases emphasize the importance of the measures implemented to limit human exposure to these potentially zoonotic agents, and of continued surveillance for both animal and human prion diseases.

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Section: Classical Bse and Variant Cjdsupporting

confidence: 65%

Animal prion diseases: the risks to human health

Houston

Andréoletti

2019

Brain Pathology

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“…Tissues were fixed in 10% formalin for processing for microscopic examination. Neuropathological methods and immunohistochemical detection of pathological prion protein (PrP sc ) were performed on brain sections as previously described 56 . Briefly, PrP was detected using the Discovery Automated IHC Stainer.…”

Section: Methodsmentioning

confidence: 99%

Transmission of scrapie prions to primate after an extended silent incubation period

Comoy

Mikol

Luccantoni-Freire

et al. 2015

Sci Rep

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Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.

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“…Even decades later, despite the growing importance of genetically modified mouse models, which provided increased versatility and better readout, primates continued to have a role in transmission studies: transmission of brain extracts from cattle with Bovine Spongiform Encephalopathy (BSE) into macaques established the first pathogenetic link between BSE and variant CJD (vCJD) [101], by producing a pathological phenotype that was nearly identical to vCJD. In more recent studies, primate models are used in correlative studies looking at transmission properties of different prion strains [135], to establish the zoonotic potential of transmissible spongiform encephalopathies (TSE) other than BSE, such as natural scrapie [46], to assess transfusion safety through application of specifically designed prion removal filters [104], or to estimate the risk of oral infection with the BSE agent [100]. Cynomolgus macaque monkey ( Macaca fascicularis ) has remained a relatively popular choice for prion research in primates, due to their longevity, phylogenetic proximity to humans, similar digestive physiology, and their genetic similarity to humans, including homozygosity for methionine at PRNP codon 129.…”

Section: Animal Models Of Prion Disease: From Monkeys To Fliesmentioning

confidence: 99%

Prion disease: experimental models and reality

Brandner

Jaunmuktane

2017

Acta Neuropathol

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overview of the use of models of prion disease, how they have evolved alongside the scientific questions, and how advancements in technologies have pushed the boundaries of our understanding of prion biology. AbstractThe understanding of the pathogenesis and mechanisms of diseases requires a multidisciplinary approach, involving clinical observation, correlation to pathological processes, and modelling of disease mechanisms. It is an inherent challenge, and arguably impossible to generate model systems that can faithfully recapitulate all aspects of human disease. It is, therefore, important to be aware of the potentials and also the limitations of specific model systems. Model systems are usually designed to recapitulate only specific aspects of the disease, such as a pathological phenotype, a pathomechanism, or to test a hypothesis. Here, we evaluate and discuss model systems that were generated to understand clinical, pathological, genetic, biochemical, and epidemiological aspects of prion diseases. Whilst clinical research and studies on human tissue are an essential component of prion research, much of the understanding of the mechanisms governing transmission, replication, and toxicity comes from in vitro and in vivo studies. As with other neurodegenerative diseases caused by protein misfolding, the pathogenesis of prion disease is complex, full of conundra and contradictions. We will give here a historical Electronic supplementary material The online version of this article

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Evaluation of the protection of primates transfused with variant Creutzfeldt‐Jakob disease–infected blood products filtered with prion removal devices: a 5‐year update

Abstract: After 5 to 6 years of progress, this ongoing study provides encouraging results on the prion blood removal performances of the P-Capt filter in macaques, an utmost relevant model for human prion diseases.

Cited by 14 publications

References 25 publications

Animal prion diseases: the risks to human health

Animal prion diseases: the risks to human health

Transmission of scrapie prions to primate after an extended silent incubation period

Prion disease: experimental models and reality

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