Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice

Griffiths, Peter C.; Spiropoulos, John; Lockey, Richard; Tout, Anna C.; Jayasena, Dhanushka; Plater, Jane M.; Chave, Alun; Green, Robert B.; Simonini, Sarah; Thorne, Leigh; Dexter, Ian; Balkema‐Buschmann, Anne; Groschup, Martin H.; Béringue, Vincent; Dur, Annick Le; Laude, Hubert; Hope, James

doi:10.1099/vir.0.018986-0

Cited by 37 publications

(62 citation statements)

References 32 publications

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“…The two atypical scrapie controls, Scr2 and 51|45, gave very similar and characteristic lesion profiles (Fig. 2d, e, respectively; type A in Table 3), particularly striking in the damage caused in the white matter area W3 (basal cerebral peduncle), as expected (Griffiths et al, 2010). Of the unusual scrapie transmissions, the vacuolar profile of L4824 ( Fig.…”

Section: Selection Of Candidate Atypical Scrapie Casesmentioning

confidence: 60%

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Chong

Kennedy

Goldmann

et al. 2015

Journal of General Virology

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Section: Selection Of Candidate Atypical Scrapie Casesmentioning

confidence: 60%

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Chong

Kennedy

Goldmann

et al. 2015

Journal of General Virology

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“…According to this definition, classical scrapie includes several strains as various distinct phenotypes can be identified in mice [33][34][35] . In contrast every time a source of atypical scrapie has been bioassayed in mice a single phenotype has been detected suggesting that atypical scrapie is attributed to a single strain 36,37) . It must be emphasized that, particularly in rodents, the phenotypes are not simply host specific but mouse line specific 30,33,38) .…”

Section: Tse Disease and Strain Characterisationmentioning

confidence: 99%

“…There were differences in the distribution and intensity of vacuolation, the distribution and the different types of PrP Sc deposits identified by IHC, and a strikingly different Western blot profile that included four bands instead of the three bands that are usually associated with animal TSEs. This new phenotype was designated Nor98 and mouse bioassays showed that it transmits only to transgenic mice and produces a phenotype that is distinct from any classical scrapie phenotype identified in these mice 36,37) . This novel disease attracted a lot of interest as it raised serious questions regarding our knowledge of TSEs and our ability to detect them reliably, as retrospective studies showed that atypical scrapie cases preexisted but they had not been diagnosed as the tests used at the time were not optimised for detecting this disease 66,67) .…”

Section: Atypical Scrapiementioning

confidence: 99%

“…This novel disease attracted a lot of interest as it raised serious questions regarding our knowledge of TSEs and our ability to detect them reliably, as retrospective studies showed that atypical scrapie cases preexisted but they had not been diagnosed as the tests used at the time were not optimised for detecting this disease 66,67) . Subsequently atypical scrapie was studied extensively and the phenotype of the disease in the original host 31,68,69) and in transgenic mice 36,37) was described in detail. Knowledge of the distribution of the agent in the nervous system in these cases led to the suggestion that during diagnostic sampling the cerebellum should be collected in addition to the obex.…”

Section: Atypical Scrapiementioning

confidence: 99%

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Pathology of Animal Transmissible Spongiform Encephalopathies (TSEs)

Spiropoulos

Simmons

2017

Food Safety

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Pathology is the study of the structural and functional changes produced by diseases or − more specifically − the lesions they cause. To achieve this pathologists employ various approaches. These include description of lesions that are visible to the naked eye which are the subject of anatomic pathology and changes at the cellular level that are visible under the microscope, the subject of histopathology. Changes at the molecular level which are identified by probes that target specific molecules − mainly proteins that are detected using immunohistochemistry (IHC). As transmissible spongiform encephalopathies (TSEs) do not cause visible lesions anatomic pathology is not applicable to their study. For decades the application of histopathology to detect vacuoles or plaques was the only means of confirming TSE disease. The subsequent discovery of the cellular prion protein (PrP C ) and its pathogenic isoform, PrP Sc , which is a ubiquitous marker of TSEs, led to the production of anti-PrP antibodies, and enabled the development of PrP Sc detection techniques such as immunohistochemistry, Histoblot and PETblot that have evolved in parallel with similar biochemical methods such as Western blot and ELISA. These methods offer greater sensitivity than histopathology in TSE diagnosis and crucially they can be applied to analyze various phenotypic aspects of single TSE sources increasing the amount of data and offering higher discriminatory power. The above principles are applied to diagnose and define TSE phenotypes which form the basis of strain characterisation.

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“…In contrast to Classical scrapie, no strain variability has been observed so far among isolates of Atypical scrapie (Le Dur et al, 2005;Griffiths et al, 2010).…”

Section: Agent Strains In Sheepmentioning

confidence: 99%

Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions

2015

EFSA Journal

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The factors that modulate the transmissibility of Transmissible Spongiform Encephalopathies (TSE) and the approaches for the study of their zoonotic potential are reviewed. The paper 'Evidence for zoonotic potential of ovine scrapie prions' by Cassard et al. (2014) is scientifically appraised, focussing on the experimental design, the results and the conclusions. The paper provides evidence in a laboratory experiment that some Classical scrapie isolates can propagate in humanised transgenic mice and produce prions that on second passage are similar to those causing one form of sporadic Creutzfeldt-Jakob disease (sCJD). It is concluded that the results from the study raise the possibility that scrapie prions have the potential to be zoonotic, but do not provide evidence that transmission can or does take place under field conditions. The conclusions of the 2011 ECDC-EFSA 'Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans' are reviewed in the light of the new scientific evidence available since its publication. This supports and strengthens the conclusions of that opinion with regard to the potential for some animal TSE to be zoonotic, but does not provide evidence of a causal link between Classical or Atypical scrapie and human TSE. Current evidence does not establish this link, and no consistent risk factors have been identified for sCJD. The possibility of scrapie-related public health risks from the consumption of ovine products cannot be assessed. Recommendations are formulated on further studies and data that are needed to investigate the zoonotic potential of animal TSE and to estimate the amount of infectivity from TSE-infected products sourced from small ruminants and entering the food chain in the European Union. © European Food Safety Authority, 2015Keywords: Atypical scrapie, Classical scrapie, Creutzfeldt-Jakob disease, transmissible spongiform encephalopathy, zoonosis Amendment: An amendment has been made to the following sentence on p. 31: 'In this regard the emergence of sCJD is not so surprising, and has been described after inoculation of tgHu mice with cattle BSE and human vCJD (Asante et al., 2002;Beringue et al., 2008b)'. This was carried out to clarify that one of the references reported emergence of sporadic CJD after experimental inoculation of humanised transgenic mice with vCJD, which was not correctly stated in the published opinion. An amendment has been made on p. 28, to add the word 'OR' between two search terms within the literature search string reported. Reproduction is authorised provided the source is acknowledged. Requestor: European CommissionThe EFSA Journal is a publication of the European Food Safety Authority, an agency of the European Union. SummaryFollowing a request from the European Commission (EC), the EFSA Panel on Biological Hazards (BIOHAZ Panel) was asked to deliver a scientific opinion on the review of a scientific publication concerning the zoonotic potential of ovine scrapie prions.T...

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Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice

Cited by 37 publications

References 32 publications

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Pathology of Animal Transmissible Spongiform Encephalopathies (TSEs)

Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions

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