Bovine spongiform encephalopathy (BSE) may have transmitted to sheep through feed and pose a risk to human health. Sheep BSE cannot be clinically distinguished from scrapie, and conventional strain typing would be impractical on a significant scale. As human prion strains can be distinguished by differences in prion protein (PrPsc) conformation and glycosylation we have applied PrP(Sc) typing to sheep. We found multiple Western blot patterns of PrP(Sc) in scrapie, consistent with the known scrapie strain diversity in sheep. Sheep passaged BSE showed a PrP(Sc) banding pattern similar to BSE passaged in other species [Collinge, J., Sidle, K.C.L., Meads, J., Ironside, J. and Hill, A.F., Nature, 383 (1996) 685-690], both in terms of fragment size following proteinase K cleavage and abundance of diglycosylated PrP. However, none of the historical or contemporary scrapie cases studied had a PrP(Sc) type identical to sheep BSE. While more extensive studies, including sheep of all PrP genotypes, will be required to fully evaluate these findings, these results suggest that large scale screening of sheep for BSE may be possible.
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http://dx.doi.org/10.1016/s0304-3940(98)00736-8 | DOI Listing |
This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (-3%, compared with 2022), with five atypical BSE cases reported (four H-type: two in Spain, one in France and one in Ireland; one L-type in the Netherlands); and 46,096 cattle by eight non-EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1).
View Article and Find Full Text PDFFront Vet Sci
October 2024
Research Centre for Encephalopathies and Transmissible Emerging Diseases. Institute for Health Research Aragón (IIS) - WOAH Reference Laboratory for BSE and Scrapie, University of Zaragoza, Zaragoza, Spain.
Introduction: According to the neuroinflammatory hypothesis, a cytokine-mediated host innate immune response may be involved in the mechanisms that contribute to the process of neurodegeneration. Specifically, regarding prion diseases, some experimental murine models have evidenced an altered profile of inflammatory intermediaries. However, the local inflammatory response has rarely been assessed, and never in tissues from different natural models throughout the progression of neurodegeneration.
View Article and Find Full Text PDFJ Infect Dis
July 2024
Université Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Université Versailles-Saint Quentin, Unité de Virologie Immunologie Moléculaires, Jouy-en-Josas, France.
Background: Atypical/Nor98 scrapie (AS) is an idiopathic infectious prion disease affecting sheep and goats. Recent findings suggest that zoonotic prions from classical bovine spongiform encephalopathy (C-BSE) may copropagate with atypical/Nor98 prions in AS sheep brains. Investigating the risk AS poses to humans is crucial.
View Article and Find Full Text PDFThe European Commission requested an estimation of the BSE risk (C-, L- and H-BSE) from gelatine and collagen derived from ovine, caprine or bovine bones, and produced in accordance with Regulation (EC) No 853/2004, or Regulation (EC) No 1069/2009 and its implementing Regulation (EU) No 142/2011. A quantitative risk assessment was developed to estimate the BSE infectivity, measured in cattle oral infectious dose 50 (CoID), in a small size batch of gelatine including one BSE-infected bovine or ovine animal at the clinical stage. The model was built on a scenario where all ruminant bones could be used for the production of gelatine and high-infectivity tissues remained attached to the skull (brain) and vertebral column (spinal cord).
View Article and Find Full Text PDFPeerJ
July 2024
Natural Science Research Laboratory, Museum of Texas Tech University, Lubbock, TX, United States of America.
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