scholarly journals Resistance of Domestic Cats to a US Sheep Scrapie Agent by Intracerebral Route

2002 ◽  
Vol 14 (5) ◽  
pp. 444-445 ◽  
Author(s):  
Amir N. Hamir ◽  
Wilber W. Clark ◽  
Diane L. Sutton ◽  
Janice M. Miller ◽  
Mick J. Stack ◽  
...  
Keyword(s):  
Western Blot ◽  
Bovine Spongiform Encephalopathy ◽  
Extended Period ◽  
Spongiform Encephalopathy ◽  
Neurologic Disease ◽  
Domestic Cats ◽  
Species Barrier ◽  
Scrapie Agent ◽  
Abnormal Form ◽  
Sheep Scrapie

Feline spongiform encephalopathy (FSE) is thought to have resulted from consumption of food contaminated with bovine spongiform encephalopathy and the latter is believed to result from the consumption of food contaminated with scrapie. However, no direct experimental documentation exists to indicate that the scrapie agent is capable of amplifying in cats, and, therefore, crossing the species barrier. During 1979, 6 cats ranging in age from 3.5 to 18 months were intracerebrally inoculated with sheep scrapie (inoculum G-639-PP) and were observed for an extended period. Inoculated cats did not develop neurologic disease, and microscopic lesions of spongiform encephalopathy were not evident. Immunohistochemistry and Western blot techniques failed to detect the abnormal form of prion protein (PrPres). These results indicate that the sheep scrapie agent (G-639-PP) used in this study was not capable of amplifying in cats and therefore was unable to cross the species barrier to produce FSE.

The neuropathologic phenotype of experimental ovine BSE is maintained after blood transfusion

Blood ◽  
2006 ◽  
Vol 108 (2) ◽  
pp. 745-748 ◽  
Author(s):  
Sílvia Sisó ◽  
Lorenzo González ◽  
Fiona Houston ◽  
Nora Hunter ◽  
Stuart Martin ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Bovine Spongiform Encephalopathy ◽  
Prion Diseases ◽  
Spongiform Encephalopathy ◽  
Neurologic Disease ◽  
Infected Blood ◽  
Route Of Infection ◽  
Jakob Disease ◽  
Iatrogenic Transmission ◽  
Sheep Scrapie

Iatrogenic transmission by blood transfusion has been described in cases of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bovine spongiform encephalopathy (BSE), and natural sheep scrapie, demonstrating that blood in these prion diseases is infectious. However, the possible effect of the transfusion, derived from differences in the inoculum (blood) and the route of infection (intravenous), on the pathologic phenotype of the disease in the recipients is not known. This study describes the neuropathologic phenotype of PrPd accumulation in sheep succumbing to neurologic disease after blood transfusion from donors experimentally infected with BSE; these were either clinically or subclinically affected at the time of donation. We demonstrate that blood can become infectious at early stages of ovine BSE infection and that the PrPd immunohistochemical phenotype is maintained after transfusion. This suggests that a change in the pathologic phenotype of vCJD would not be expected as a result of exposure to infected blood.

scholarly journals Sheep-Passaged Bovine Spongiform Encephalopathy Agent Exhibits Altered Pathobiological Properties in Bovine-PrP Transgenic Mice

2006 ◽  
Vol 81 (2) ◽  
pp. 835-843 ◽  
Author(s):  
Juan Carlos Espinosa ◽  
Olivier Andréoletti ◽  
Joaquín Castilla ◽  
María Eugenia Herva ◽  
Mónica Morales ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Clinical Signs ◽  
Health Concern ◽  
Mouse Bioassay ◽  
Spongiform Encephalopathy ◽  
Species Barrier ◽  
Subsequent Passage ◽  
Sheep Scrapie ◽  
Cattle And Sheep ◽  
Transmission Barrier

ABSTRACT Sheep can be experimentally infected with bovine spongiform encephalopathy (BSE), and the ensuing disease is similar to scrapie in terms of pathogenesis and clinical signs. BSE infection in sheep is an animal and human health concern. In this study, the transmission in BoPrP-Tg110 mice of prions from BSE-infected sheep was examined and compared to the transmission of original cattle BSE in cattle and sheep scrapie prions. Our results indicate no transmission barrier for sheep BSE prions to infect BoPrP-Tg110 mice, but the course of the disease is accelerated compared to the effects of the original BSE isolate. The shortened incubation period of sheep BSE in the model was conserved in subsequent passage in BoPrP-Tg110 mice, indicating that it is not related to infectious titer differences. Biochemical signature, lesion profile, and PrPSc deposition pattern of both cattle and sheep BSE were similar. In contrast, all three sheep scrapie isolates tested showed an evident transmission barrier and further adaptation in subsequent passage. Taken together, those data indicate that BSE agent can be altered by crossing a species barrier, raising concerns about the virulence of this new prion towards other species, including humans. The BoPrP-Tg110 mouse bioassay should be considered as a valuable tool for discriminating scrapie and BSE in sheep.

scholarly journals Molecular Analysis of the Abnormal Prion Protein during Coinfection of Mice by Bovine Spongiform Encephalopathy and a Scrapie Agent

2001 ◽  
Vol 75 (1) ◽  
pp. 107-114 ◽  
Author(s):  
Thierry G. M. Baron ◽  
Anne-Gaelle Biacabe
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Proteinase K ◽  
Spongiform Encephalopathy ◽  
Molecular Features ◽  
Electrophoretic Mobilities ◽  
Scrapie Agent ◽  
Scrapie Strain ◽  
Sheep Scrapie

ABSTRACT Molecular features of the proteinase K-resistant prion protein (PrP res) may discriminate among prion strains, and a specific signature could be found during infection by the infectious agent causing bovine spongiform encephalopathy (BSE). To investigate the molecular basis of BSE adaptation and selection, we established a model of coinfection of mice by both BSE and a sheep scrapie strain (C506M3). We now show that the PrP res features in these mice, characterized by glycoform ratios and electrophoretic mobilities, may be undistinguishable from those found in mice infected with scrapie only, including when mice were inoculated by both strains at the same time and by the same intracerebral inoculation route. Western blot analysis using different antibodies against sequences near the putative N-terminal end of PrP res also demonstrated differences in the main proteinase K cleavage sites between mice showing either the BSE or scrapie PrP res profile. These results, which may be linked to higher levels of PrP res associated with infection by scrapie, were similar following a challenge by a higher dose of the BSE agent during coinfection by both strains intracerebrally. Whereas PrP res extraction methods used allowed us to distinguish type 1 and type 2 PrP res, differing, like BSE and scrapie, by their electrophoretic mobilities, in the same brain region of some patients with Creutzfeldt-Jakob disease, analysis of in vitro mixtures of BSE and scrapie brain homogenates did not allow us to distinguish BSE and scrapie PrP res. These results suggest that the BSE agent, the origin of which remains unknown so far but which may have arisen from a sheep scrapie agent, may be hidden by a scrapie strain during attempts to identify it by molecular studies and following transmission of the disease in mice.

scholarly journals A Comparison of Rapid Bovine Spongiform Encephalopathy Testing Methods on Autolyzed Bovine Brain Tissue

2005 ◽  
Vol 17 (2) ◽  
pp. 99-102 ◽  
Author(s):  
Angus Wear ◽  
Kirstine Henderson ◽  
Kath Webster ◽  
Indu Patel
Keyword(s):  
Western Blot ◽  
Bovine Spongiform Encephalopathy ◽  
Bovine Brain ◽  
Spongiform Encephalopathy ◽  
The European Union ◽  
Routine Testing ◽  
New Methods ◽  
Surveillance Programs ◽  
The Eu ◽  
Do So

In 1999, the European Union (EU) approved 3 rapid methods for the testing of bovine brain samples for the presence of bovine spongiform encephalopathy (BSE). The evaluation that led to the approval did not include an analysis of autolyzed material. Member states of the EU have active surveillance programs for BSE, which target fallen stock as well as other categories of cattle. Autolysis is a common feature of fallen stock samples because there can be a considerable delay between death and collection of samples. Therefore, it is important to know whether these tests perform optimally on autolyzed samples. The Veterinary Laboratories Agency (VLA) selected 250 positive fallen stock samples. These had been detected during routine testing using the Prionics®-Check Western blot and confirmed as BSE cases by immunohistochemistry or electron microscopy. Samples were graded according to the degree of autolysis and then tested by the 3 methods: Prionics®-Check Western blot, Platelia test, and Enfer test. All 3 methods correctly classified the samples as positive BSE cases, therefore alleviating doubt about their ability to do so. Subsequent EU validation exercises, such as those conducted in 2002–2003, have included the testing of autolyzed material. It is important that all new methods be evaluated on autolyzed tissue before approval for official use.

Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier

1994 ◽  
Vol 343 (1306) ◽  
pp. 405-411 ◽  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Interspecies Transmission ◽  
Spongiform Encephalopathy ◽  
Species Barrier ◽  
New Host ◽  
Disease Characteristics ◽  
Low Efficiency ◽  
Specific Influence ◽  
Donor Species ◽  
Selection Of

Transmissions of bovine spongiform encephalopathy (BSE) from seven unrelated cattle sources have given remarkably uniform disease characteristics in mice, differing from over twenty previous and contemporary transmissions of sheep and goat scrapie. Transmissions to mice of spongiform encephalopathy from six species (including sheep and goats) which have been experimentally or naturally infected with bse have given similar results to direct BSE transmissions from cattle. Therefore the BSE agent has retained its identity when passaged through a range of species and the ‘donor’ species has little specific influence on disease characteristics in mice, adding to evidence for an agent-specific informational molecule. On transmission of BSE or scrapie to mice the incubation periods are long compared with subsequent mouse-to-mouse passages (the ‘species barrier’). C ontributing factors include a low efficiency of infection on interspecies transmission, the apparent failure of intracerebrally injected ‘foreign’ inoculum to establish infection directly in mouse brain and the selection of variant strains of agent which replicate most readily in the new host species.

Zombie deer and the species barrier. Should humans worry about it?

2020 ◽  
Keyword(s):  
Chronic Disease ◽  
Bovine Spongiform Encephalopathy ◽  
Chronic Wasting Disease ◽  
Spongiform Encephalopathy ◽  
Species Barrier ◽  
Wasting Disease ◽  
Prion Infection ◽  
The Moment

This commentary reports of a deer chronic disease (chronic wasting disease - CWD), which might be transmitted to humans. It is due to a prion infection, similar to the bovine spongiform encephalopathy (BSE). At the moment, it is not known if the disease may be transmitted to humans. That is why all of us should be aware of the disease, and more careful while consuming deer meat.

scholarly journals Infectious titres of sheep scrapie and bovine spongiform encephalopathy agents cannot be accurately predicted from quantitative laboratory test results

2012 ◽  
Vol 93 (11) ◽  
pp. 2518-2527 ◽  
Author(s):  
Lorenzo González ◽  
Leigh Thorne ◽  
Martin Jeffrey ◽  
Stuart Martin ◽  
John Spiropoulos ◽  
...  
Keyword(s):  
Western Blot ◽  
Bovine Spongiform Encephalopathy ◽  
Surrogate Marker ◽  
Inverse Correlation ◽  
Transmissible Spongiform Encephalopathies ◽  
Spongiform Encephalopathy ◽  
Test Results ◽  
Biochemical Tests ◽  
Spongiform Encephalopathies ◽  
Laboratory Test Results

It is widely accepted that abnormal forms of the prion protein (PrP) are the best surrogate marker for the infectious agent of prion diseases and, in practice, the detection of such disease-associated (PrPd) and/or protease-resistant (PrPres) forms of PrP is the cornerstone of diagnosis and surveillance of the transmissible spongiform encephalopathies (TSEs). Nevertheless, some studies question the consistent association between infectivity and abnormal PrP detection. To address this discrepancy, 11 brain samples of sheep affected with natural scrapie or experimental bovine spongiform encephalopathy were selected on the basis of the magnitude and predominant types of PrPd accumulation, as shown by immunohistochemical (IHC) examination; contra-lateral hemi-brain samples were inoculated at three different dilutions into transgenic mice overexpressing ovine PrP and were also subjected to quantitative analysis by three biochemical tests (BCTs). Six samples gave ‘low’ infectious titres (106.5 to 106.7 LD50 g−1) and five gave ‘high titres’ (108.1 to ≥108.7 LD50 g−1) and, with the exception of the Western blot analysis, those two groups tended to correspond with samples with lower PrPd/PrPres results by IHC/BCTs. However, no statistical association could be confirmed due to high individual sample variability. It is concluded that although detection of abnormal forms of PrP by laboratory methods remains useful to confirm TSE infection, infectivity titres cannot be predicted from quantitative test results, at least for the TSE sources and host PRNP genotypes used in this study. Furthermore, the near inverse correlation between infectious titres and Western blot results (high protease pre-treatment) argues for a dissociation between infectivity and PrPres.

scholarly journals Three serial passages of bovine spongiform encephalopathy in sheep do not significantly affect discriminatory test results

2009 ◽  
Vol 90 (3) ◽  
pp. 764-768 ◽  
Author(s):  
Michael Stack ◽  
Lorenzo González ◽  
Martin Jeffrey ◽  
Stuart Martin ◽  
Colin Macaldowie ◽  
...  
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Proteins ◽  
Oral Exposure ◽  
Spongiform Encephalopathy ◽  
Test Results ◽  
Western Immunoblotting ◽  
Jakob Disease ◽  
Disease Variant ◽  
The Uk ◽  
Sheep Scrapie

During the 1980s, bovine spongiform encephalopathy (BSE)-contaminated meat and bonemeal were probably fed to sheep, raising concerns that BSE may have been transmitted to sheep in the UK. The human disease, variant Creutzfeldt–Jakob disease, arose during the BSE epidemic, and oral exposure of humans to BSE-infected tissues has been implicated in its aetiology. The concern is that sheep BSE could provide another source of BSE exposure to humans via sheep products. Two immunological techniques, Western immunoblotting (WB) and immunohistochemistry (IHC), have been developed to distinguish scrapie from cases of experimental sheep BSE by the characteristics of their respective abnormal, disease-associated prion proteins (PrPd). This study compares the WB and IHC characteristics of PrPd from brains of primary, secondary and tertiary experimental ovine BSE cases with those of cattle BSE and natural sheep scrapie. Discrimination between experimental sheep BSE and scrapie remained possible by both methods, regardless of the route of challenge.

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