Amyloidosis: The Key to the Epidemiology and Pathogenesis of Transmissible Spongiform Encephalopathies

Diringer, Heino; Beekes, Michael; Baldauf, Elizabeth; Cassens, Sven; Özel, Muhsin

doi:10.1007/978-1-4612-2406-8_18

Cited by 2 publications

(2 citation statements)

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“…Based in part on the interspecies transmission observed in animal TSEs, Diringer (1996) hypothesised a link between familial CJD and scrapie, but a comparison of the number of sheep per human population scrapie prevalence and mortality rates for sporadic CJD, published in response, argued that the incidence of sporadic CJD was independent of exposure of the human population to sheep and scrapie . The epidemiological situation has not changed and an update of the worldwide occurrence of the diseases (i.e.…”

Section: Epidemiological Approachmentioning

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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Section: Epidemiological Approachmentioning

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

View full text Add to dashboard Cite

show abstract

“…Based in part on the interspecies transmission observed in animal TSEs, Diringer (1996) hypothesised a link between familial CJD and scrapie, but a comparison of the number of sheep per human population scrapie prevalence and mortality rates for sporadic CJD, published in response, argued that the incidence of sporadic CJD was independent of exposure of the human population to sheep and scrapie (Will et al, 1996). The epidemiological situation has not changed and an update of the worldwide occurrence of the diseases (i.e.…”

Section: Epidemiological Approachmentioning

confidence: 99%

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2015

EFSA Journal

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Amyloidosis: The Key to the Epidemiology and Pathogenesis of Transmissible Spongiform Encephalopathies

Cited by 2 publications

References 72 publications

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

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

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