Experimental transmission of ovine atypical scrapie to cattle

Konold, Timm; Spiropoulos, John; Hills, Janet; Abdul, Hasina; Cawthraw, Saira; Phelan, Laura; McKenna, Amy; Read, Lauren; Canoyra, Sara; Marín-Moreno, Alba; Torres, Juan María

doi:10.1186/s13567-023-01224-3

Cited by 3 publications

(1 citation statement)

References 52 publications

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“…In the mid-1980s, a large epidemic of BSE-C emerged in the UK and its spread was rapidly linked to the recycling and use of meat and bone meals (MBM) containing TSE-infected bovine material as feed additives within the ruminant feed chain. Recent evidence links atypical scrapie as a plausible origin for the epidemic [ 20 – 23 ]. Ten years later, frightening evidence was published linking the consumption of BSE-C contaminated material to a new human prion disease, called variant CJD (vCJD) [ 24 , 25 ] that has been reported to have killed 224 people, mainly in the UK [ 26 ], and is thus the only zoonotic prion recognized to date.…”

Section: Introductionmentioning

confidence: 99%

Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents

Marín-Moreno,

Benestad,

Barrio

et al. 2024

Vet Res

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The first case of CWD in a Norwegian red deer was detected by a routine ELISA test and confirmed by western blotting and immunohistochemistry in the brain stem of the animal. Two different western blotting tests were conducted independently in two different laboratories, showing that the red deer glycoprofile was different from the Norwegian CWD reindeer and CWD moose and from North American CWD. The isolate showed nevertheless features similar to the classical BSE (BSE-C) strain. Furthermore, BSE-C could not be excluded based on the PrPSc immunohistochemistry staining in the brainstem and the absence of detectable PrPSc in the lymphoid tissues. Because of the known ability of BSE-C to cross species barriers as well as its zoonotic potential, the CWD red deer isolate was submitted to the EURL Strain Typing Expert Group (STEG) as a BSE-C suspect for further investigation. In addition, different strain typing in vivo and in vitro strategies aiming at identifying the BSE-C strain in the red deer isolate were performed independently in three research groups and BSE-C was not found in it. These results suggest that the Norwegian CWD red deer case was infected with a previously unknown CWD type and further investigation is needed to determine the characteristics of this potential new CWD strain.

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Section: Introductionmentioning

confidence: 99%

Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents

Marín-Moreno,

Benestad,

Barrio

et al. 2024

Vet Res

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Assessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions

Marín-Moreno,

Reine,

Herzog

et al. 2024

The Journal of Infectious Diseases

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Background Atypical/Nor98 scrapie (AS) is an idiopathic infectious prion disease affecting sheep and goats. Recent findings suggest that zoonotic prions from bovine spongiform encephalopathy (C-BSE) may co-propagate with atypical/Nor98 prions in AS sheep brains. Investigating the risk AS poses to humans is crucial. Methods To assess the risk of sheep/goat-to-human transmission of AS, we serially inoculated brain tissue from field and laboratory isolates into transgenic mice overexpressing human prion protein (Met129 allele). We studied clinical outcomes as well as presence of prions in brains and spleens. Results No transmission occurred on the primary passage, with no clinical disease or pathological prion protein in brains and spleens. On subsequent passages, one isolate gradually adapted, manifesting as prions with a phenotype resembling those causing MM1-type sporadic Creutzfeldt-Jakob disease in humans. However, further characterization using in vivo and in vitro techniques confirmed both prion agents as different strains, revealing a case of phenotypic convergence. Importantly, no C-BSE prions emerged in these mice, especially in the spleen, which is more permissive than the brain for C-BSE cross-species transmission. Conclusions The results obtained suggest a low the zoonotic for AS. Rare adaptation may allow the emergence of prions phenotypically resembling those spontaneously forming in humans.

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Transmission of Norwegian reindeer CWD to sheep by intracerebral inoculation results in an unusual phenotype and prion distribution

Harpaz,

Cazzaniga,

Tran

et al. 2024

Vet Res

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Chronic wasting disease (CWD), a prion disease affecting cervids, has been known in North America (NA) since the 1960s and emerged in Norway in 2016. Surveillance and studies have revealed that there are different forms of CWD in Fennoscandia: contagious CWD in Norwegian reindeer and sporadic CWD in moose and red deer. Experimental studies have demonstrated that NA CWD prions can infect various species, but thus far, there have been no reports of natural transmission to non-cervid species. In vitro and laboratory animal studies of the Norwegian CWD strains suggest that these strains are different from the NA strains. In this work, we describe the intracerebral transmission of reindeer CWD to six scrapie-susceptible sheep. Detection methods included immunohistochemistry (IHC), western blot (WB), enzyme-linked immunosorbent assay (ELISA), real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA). In the brain, grey matter vacuolation was limited, while all sheep exhibited vacuolation of the white matter. IHC and WB conventional detection techniques failed to detect prions; however, positive seeding activity with the RT-QuIC and PMCA amplification techniques was observed in the central nervous system of all but one sheep. Prions were robustly amplified in the lymph nodes of all animals, mainly by RT-QuIC. Additionally, two lymph nodes were positive by WB, and one was positive by ELISA. These findings suggest that sheep can propagate reindeer CWD prions after intracerebral inoculation, resulting in an unusual disease phenotype and prion distribution with a low amount of detectable prions.

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Experimental transmission of ovine atypical scrapie to cattle

Cited by 3 publications

References 52 publications

Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents

Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents

Assessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions

Transmission of Norwegian reindeer CWD to sheep by intracerebral inoculation results in an unusual phenotype and prion distribution

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