Classical Bovine Spongiform Encephalopathy by Transmission of H-Type Prion in Homologous Prion Protein Context

Torres, Juan María; Andréoletti, Olivier; Lacroux, Caroline; Prieto, Irene; Lorenzo, Piera Di; Larska, Magdalena; Baron, Thierry; Espinosa, Juan Carlos

doi:10.3201/eid1709.101403

Cited by 60 publications

(68 citation statements)

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“…Commercially available rapid immunochemical tests for PrP D can, in the best cases, give positive responses from 10 Ϫ3 to 10 Ϫ4 dilutions of postmortem brain tissues with high levels of PrP D (33, 34). Immunoblotting for PrP Res can detect BSE-infected tissues with similar sensitivity and also discriminate between the bovine strains based on the relative electrophoretic migration and glycoform ratios of the PrP Res bands (19)(20)(21)(22). A more sensitive conformation-dependent immunoassay (CDI) has also been described for C-BSE, which has a sensitivity similar to that of endpoint dilution bioassays in a line of transgenic mice expressing bovine PrP [Tg(BoPrP ϩ/ϩ )4092-Prnp 0/0 ], that is, with 50% positive responses at C-BSE brain dilutions of ϳ10 Ϫ5 (35).…”

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Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

et al. 2016

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Many mammalian species can be afflicted with prion diseases or transmissible spongiform encephalopathies. Prion diseases are neurodegenerative, transmissible, untreatable, and fatal (reviewed in references 1 and 2). The underlying pathogenesis of prion diseases involves the conversion of the host's normal prion protein, PrP C , to abnormal forms that are usually more protease resistant, multimeric, and insoluble. The multimeric and insoluble forms have been generically called PrP Sc (PrP-scrapie), or more functionally PrP Res (protease resistant) and PrP D (disease associated). At least some of these forms comprise the transmissible agent or prion (3-10). Multiple prion strains can be propagated within a given host species, giving consistently different clinical, pathological, and molecular phenotypes (11-16).The first prion disease to be recognized in cattle was classical bovine spongiform encephalopathy (C-BSE). C-BSE was likely caused primarily by widespread prion contamination of cattle feed (17). After peaking in the early 1990s, the incidence of C-BSE has now been greatly reduced by regulatory measures that limit its horizontal spread. C-BSE is the only known zoonotic prion disease, having caused variant Creutzfeldt-Jakob disease (vCJD) in humans who presumably consumed contaminated beef. Although new clinical cases of vCJD are rare (http://www.cjd.ed.ac .uk/documents/figs.pdf), a recent survey of appendices in the United Kingdom suggests a high incidence of subclinical vCJD infections, at ϳ1:2,000 of the population born between 1941 and 1985 (18).Since the C-BSE epidemic, two atypical strains of BSE, H-type BSE (H-BSE) and L-type BSE (L-BSE), have also been identified in cattle. PrPRes is usually composed of a mixture of glycosylated and unglycosylated molecules, and the various BSE strains can be differentiated biochemically using Western blotting of postmortem brain tissue samples by comparing the proteinase K (PK)-treated PrPRes banding patterns (19)(20)(21)(22). The H and L types of BSE are classified by their respective high and low apparent molecular masses of the unglycosylated PrP Res band. These atypical BSE strains, which are rare (Ͻ100 cases identified worldwide), tend to affect older animals (23) and appear to represent sporadic forms of bovine prion diseases (24). Despite the apparent rarity of the various types of BSE, the facts that H-and L-BSE appear to arise spontaneously and have distinct transmissibilities (20, 25-32) make it important to be able to detect and differentiate them to reduce the risk of transmission to cattle or other species, such as humans.Several in vitro methods have been developed to detect C-, L-, and H-BSE. Commercially available rapid immunochemical tests for PrP D can, in the best cases, give positive responses from 10 Ϫ3 to 10 Ϫ4 dilutions of postmortem brain tissues with high levels of PrP D (33, 34). Immunoblotting for PrP Res can detect BSE-infected tissues with similar sensitivity and also discriminate between the bovine strains based on the relative electro...

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“…Res banding patterns (19)(20)(21)(22). The H and L types of BSE are classified by their respective high and low apparent molecular masses of the unglycosylated PrP Res band.…”

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Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

et al. 2016

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“…Several studies have shown that all three BSE agents are efficiently transmitted to these transgenic mice, showing neuropathological and molecular phenotypes that are distinct for each BSE agent (Beringue et al, 2006;Buschmann et al, 2006;Beringue et al, 2007;Capobianco et al, 2007;Okada et al, 2010;Torres et al, 2011). In all cases, all three BSE agents were fully transmitted to these Tg-bov mouse models, although L-BSE showed survival times that were shorter (≈ 200 days) than H-BSE (≈ 250-300 days), but which were higher than those of C-BSE.…”

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Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE

2014

EFSA Journal

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Information on the pathogenesis and tissue distribution of Atypical Bovine Spongiform Encephalopathy (BSE) in cattle through the study of field cases and experimental transmission studies is lacking. The latter are limited to transmission of Atypical BSE through intracerebral (i.c.) inoculation of cattle. All data currently available relate to the presence or absence of PrP Sc , but do not quantify relative amounts of PrP Sc or levels of infectivity. A laboratory protocol for further studies is recommended, to allow the assessment of the relative infectious titre, PrP Sc accumulation and prion seeding activity in the tissues of cattle that developed H-BSE or L-BSE (using posterior brainstem as a reference). Tissues to be covered by those studies are categorised in three priorities, based on their inclusion in the list of specific risk material in cattle, on the presence of infectivity, or PrP Sc presence, demonstrated in Atypical BSEs or other Transmissible Spongiform Encephalopathies (TSEs) in ruminants, and on the importance in terms of input into the food chain in the EU. The protocol provides details in terms of the minimum number of animals to be tested, processing and preparation of tissues, and methods to be used to identify abnormal PrP and quantify infectivity, also depending on the expected level of infectivity and amount of tissue available for analysis. It is recommended that, through the implementation of the protocol, information should also be obtained on the performance of currently validated rapid tests for TSE active surveillance in cattle/bioassay for detecting H-BSE and L-BSE agents.

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“…De manière inattendue, de tels phénomènes évolutifs ont été mis en évidence lors de transmission homotypique, lorsque le prion et la souris transgénique expérimentalement infectée expriment la même séquence de PrP C . Par exemple, un prion identique à celui responsable de l'ESB classique a été obtenu après transmission itérative de l'ESB atypique de type H à des souris transgéniques pour la PrP bovine (Torres et al, 2011). La transmission itérative de prions vMCJ à la souris transgénique pour la PrP humaine a conduit à l'émergence, dans le cerveau, de prions identiques à ceux responsables de formes sporadiques de MCJ.…”

Section: Figure 3 : Les Souches De Prions Se Différencient Par I) Lesunclassified

Les prions, agents d’encéphalopathies transmissibles chez les mammifères

Béringue¹

2017

bavf

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Les maladies à prion sont des affections neurodégénératives d'issue fatale touchant principalement les ruminants d'élevage et l'homme. Les prions sont des agents infectieux constitués d'une protéine de l'hôte, la PrP qui, sous sa forme pathologique, s'agrège en polymères de type amyloïde autopropagés. Bien que totalement dépourvus de génome, les prions montrent des propriétés analogues à celles d'autres agents infectieux, dont la diversité de souche, le tropisme tissulaire et la capacité à franchir les barrières de transmission interspécifique. Le but de cette revue est de faire une synthèse des connaissances et des avancées récentes dans ce domaine complexe.

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Classical Bovine Spongiform Encephalopathy by Transmission of H-Type Prion in Homologous Prion Protein Context

Abstract: TOC Summary: An epidemic agent could have originated from such a cattle prion.

Cited by 60 publications

References 38 publications

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE

Les prions, agents d’encéphalopathies transmissibles chez les mammifères

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